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Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Introduction to Educational Research Connecting Methods to Practice 1st Edition
Lochmiller Test Bank
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Chapter 4 Test Bank
Multiple Choice
1. Which of these is an example of a professional organization that has established ethical standards for education researchers?
*a. American Educational Research Association
b. The U.S. Supreme Court
c. National School Boards Association
d. American Federation of Teachers
Learning Objective: 4-2
Cognitive Domain: Knowledge
Answer Location: Ethics and Educational Research
Question Type: MC
2. Ethical issues may come up _____________________ the research process.
a. only at the beginning of
b. primarily towards the end of
c. very rarely during
*d. throughout
Learning Objective: 4-1
Cognitive Domain: Comprehension
Answer Location: Ethics and Educational Research
Question Type: MC
3. Which of these is one of the primary reasons for following ethical research practices?
a. To ensure that study results get published in peer-reviewed journals
*b. To protect current and/or future research participants
c. To check if the research methodologies are reliable
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d. To establish the significance of a research study
Learning Objective: 4-1
Cognitive Domain: Application
Answer Location: Ethics and Educational Research
Question Type: MC
4. Any institution conducting research that is funded by ________________________ must have an Institutional Review Board or ethics board.
a. local donors
b. private corporations
*c. the U.S. government
d. the school board
Learning Objective: 4-2
Cognitive Domain: Comprehension
Answer Location: Ethics and Educational Research
Question Type: MC
5. Which of these does NOT describe a researcher’s ethical responsibility toward his/her research participants?
a. To protect participants from intentional or unintentional harm as a result of the research process
b. To maintain confidentiality regarding participants’ information
c. To obtain their consent throughout the research process
*d. To publish the findings in a timely manner
Learning Objective: 4-1
Cognitive Domain: Application
Answer Location: Ethics and Educational Research
Question Type: MC
6. Which of these describes one way a researcher might uphold his/her ethical responsibilities among colleagues?
*a. Inviting a colleague to review data and analysis methods on a research study to check their validity
b. Asking for informed consent
c. Quoting a colleague’s research without citing it
d. Submitting research for publication before consulting a co-author
Learning Objective: 4-1
Cognitive Domain: Analysis
Answer Location: Ethics and Educational Research
Question Type: MC
7. Imagine that you are a paid board member of an educational publishing company that designs and sells standardized testing materials for statewide reading tests, and you would like to conduct research on the reliability of these tests. What is an ethical issue that would most likely arise in this scenario?
a. Direct consent
*b. Conflict of interest
c. Institutional Review Board approval
d. Participants’ capacity
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: Ethics and Educational Research
Question Type: MC
8. If you are planning a research study that will involve interviewing teachers after school hours, how would you ask for their agreement to participate?
a. Third-party consent
b. Assent
*c. Direct consent
An Introduction to Educational Research
Publications, 2017.
Lochmiller,
Instructor Resource
SAGE Publications, 2017.
d. Informally
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
9. For which group of research participants would you need to obtain third-party consent?
*a. Third grade students
b. School administrators
c. Cafeteria staff
d. Graduate students
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
10. Which of these would NOT be included in the informed consent process?
a. Discussing the purpose of your research study
b. Outlining the types of activities participants will be asked to complete as part of the research
c. Informing participants of potential risks of the research and how they will be handled
*d. Disclosing the identities of other research participants
Learning Objective: 4-5
Cognitive Domain: Analysis
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
11. It is important to inform research participants that their consent to participate is completely___________________.
a. mandatory
b. permanent
*c. voluntary
d. risk-free
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
12. For which of these groups would you obtain direct consent for a research study?
a. Adults with cognitive disabilities
*b. Members of a professional organization
c. Kindergarten students
d. Athletes on a middle school track team
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
13. Imagine that you want to conduct research on reading instruction with a class of second grade students. Who would provide consent for research with these students?
a. Their teacher
b. Their principal
c. The students themselves
*d. The students’ parents or guardians
Learning Objective: 4-5
Cognitive Domain: Analysis
An Introduction to Educational Research
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
14. When a minor agrees to participate in a research study, this is called _________________________.
*a. assent
b. consent
c. direct consent
d. third-party consent
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
15. Which of these would NOT be considered when inviting assent from a minor for a research study?
a. The child’s age
*b. The child’s previous teacher
c. The child’s cognitive abilities
d. The institutional requirements of your school and (if applicable) the child’s school
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
16. Which of these terms refers to an individual’s ability to understand and retain information, particularly related to evaluating information about a research study and whether or not to participate?
a. Consent
b. Confidentiality
*c. Capacity
d. Vulnerability
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
17. Which of the following factors would NOT be considered in determining the capacity of a potential research participant?
a. Age
*b. Ethnicity
c. Cognitive disability
d. Emotional disability
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
18. Which of the following would be considered a vulnerable population for research purposes?
*a. Prisoners
b. Superintendents
c. College freshmen
d. Mental health professionals
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
19. Which of these describes a function of an Institutional Review Board (IRB)?
a. To conduct peer-review of research studies
b. To offer advice on a study’s theoretical framework
*c. To determine whether proposed research complies with federal and institutional regulations
d. To give guidance on conducting a literature review
Learning Objective: 4-3
Cognitive Domain: Comprehension
Answer Location: Legal and Institutional Ethical Requirements
Question Type: MC
20. Which of the following aspects of your research study would NOT likely be required to be reviewed by the Institutional Review Board?
a. Rationale for the research
b. Participant recruitment and sampling procedures
c. How data will be collected and securely stored
*d. Summaries of related literature
Learning Objective: 4-3
Cognitive Domain: Application
Answer Location: Legal and Institutional Ethical Requirements
Question Type: MC
21. Imagine that you are a graduate student who wants to conduct research in an elementary school close to your university. In addition to obtaining IRB approval for your research study from your university, what else would be necessary in order to begin your research?
*a. Determining if the school district requires an additional IRB application
b. Letting teachers know you will be gathering data
c. Collecting student data
d. Working with your faculty adviser to search for literature
Learning Objective: 4-4
Cognitive Domain: Analysis
Answer Location: Managing Relationships with Schools and Districts
Question Type: MC
22. Which of these is an important ethical consideration for the planning phase of a research study?
a. Assuring that no plagiarism has occurred in the research report
*b. Identifying and disclosing conflicts of interest
c. Making sure the data has not been fabricated
d. Crafting a strong conceptual framework
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: The Role of Ethics in Designing and Planning a Study
Question Type: MC
23. Which of these would be the BEST method for maintaining a secure dataset as you carry out your research study?
a. Keeping printed data in a folder on your classroom desk
b. Storing electronic data on an external hard drive in your home office
c. Linking to electronic data on your personal blog
*d. Keeping electronic data on a password-protected personal computer
Learning Objective: 4-5
Cognitive Domain: Analysis
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
24. Removing any identifiable characteristics from the data that could potentially identify your study’s participants is called _________________________________.
a. massaging the data
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
b. triangulating the data
*c. anonymizing the data
d. fabricating the data
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
25. Which of these is an example of how a researcher might protect research participants’ confidentiality when working with interview data?
a. Asking a graduate assistant to transcribe the interviews
*b. Applying psuedonyms to all individuals mentioned, including the interviewee
c. Performing validity checks with colleagues
d. Massaging the data
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
26. Imagine you are a principal conducting a research study with teachers on the processes for referring students for special education services within your school. Two of the teachers you interviewed shared that they felt the special education teacher did not adequately inform parents of their rights, but you don’t want to share this finding in your research report because you don’t want to upset the special education teacher. What ethical concern does this raise?
a. It is an example of fabricating the data.
b. Participants’ confidentiality is compromised.
*c. It is an example of massaging the data.
d. It is plagiarism.
Learning Objective: 4-7
Cognitive Domain: Analysis
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
27. Which of these is an example of fabricating data in a research study?
a. Using a colleague’s research without proper citation
b. Misinterpreting a quote from a research participant
c. Applying pseudonyms to interviewees
*d. Producing fieldnotes for observations that were never conducted
Learning Objective: 4-7
Cognitive Domain: Application
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
28. Which of these should NOT be a factor in determining first authorship for research projects with several collaborators?
*a. Whether an individual is a well-known scholar
b. Whether an individual was instrumental in conceptualizing the study
c. Whether an individual was actively involved in data collection and analysis
d. Whether an individual contributed significantly to writing the results/findings
Learning Objective: 4-7
Cognitive Domain: Comprehension
Answer Location: The Role of Ethics in Reporting Research Findings
Question Type: MC
29. Your faculty advisor has written several peer-reviewed articles that pertain to your research topic, and you would like to include them in your literature review. What do you need to do?
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
a. Obtain informed consent to use them
*b. Properly cite the publications when you refer to them
c. Quote lengthy passages to support your findings, but do not cite
d. Use the data from the articles in conjunction with your own to produce findings
Learning Objective: 4-7
Cognitive Domain: Analysis
Answer Location: The Role of Ethics in Reporting Research Findings
Question Type: MC
30. Which of these terms refers to using someone else’s ideas, thoughts, or words without citing or quoting them?
a. Interpretation
b. Assigning authorship
*c. Plagiarism
d. Massaging the data
Learning Objective: 4-7
Cognitive Domain: Knowledge
Answer Location: The Role of Ethics in Reporting Research Findings
Question Type: MC
31. Which of these might be an ethical consideration for conducting research using a social media website, such as Twitter?
a. Creating a Twitter account
b. Compiling the data
c. Using a qualitative data analysis software package, such as ATLAS.ti
*d. Whether the information collected is considered public or private
Learning Objective: 4-7
Cognitive Domain: Application
Answer Location: Emerging Ethical Challenges
Question Type: MC
32. You are conducting research that includes results from an online survey as part of the dataset. When you close the survey, you are able to see the email addresses from those individuals who respond, but you delete this portion of the data before you begin analysis. This data would be considered
*a. confidential
b. anonymized
c. fabricated
d. massaged
Learning Objective: 4-6
Cognitive Domain: Application
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
33. During the research process, at what point should you submit your research plans to the Institutional Review Board for review?
a. After you have collected your data
*b. Prior to beginning your research study
c. When you begin to write up your findings
d. When you submit your research report for publication
Learning Objective: 4-3
Cognitive Domain: Analysis
Answer Location: Legal and Institutional Ethical Requirements
Question Type: MC
______________________.
34. You wish to conduct a research study at a developmental learning center with a group of adults who are cognitively disabled. Which of these ethical issues would be most likely to arise as you plan for this research?
a. The age of the research participants
*b. The capacity of the research participants
c. The location of the research site
d. A conflict of interest with the research participants
Learning Objective: 4-5
Cognitive Domain: Analysis
Answer Location: Inviting Participants’ Consent and Assent
Question Type: MC
35. Education research studies are often considered to be minimally risky for participants, and are thus ______ IRB review.
a. subject to full
b. required to submit to external
c. denied
*d. exempt from
Learning Objective: 4-3
Cognitive Domain: Comprehension
Answer Location: Legal and Institutional Ethical Requirements
Question Type: MC
36. Imagine you are a school principal who has conducted research involving interviews with teachers at your school. Although this was not originally part of your research plan, you want to ask your assistant principal to listen to the interviews and help with analysis of this data. What ethical issue might arise in this situation?
*a. Participant confidentiality would be compromised.
b. This would constitute massaging the data.
c. Participants don’t have the capacity to consent to the research.
d. This is an expedited study.
Learning Objective: 4-5
Cognitive Domain: Analysis
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: MC
True/False
37. When inviting and recording informed consent from your research participants, it is important to consult Institutional Review Board guidelines for your institution.
*a. True
b. False
Learning Objective: 4-3
Cognitive Domain: Comprehension
Answer Location: Inviting Participants’ Consent and Assent
Question Type: TF
38 When a research participant consents to participate in a research study, he/she must remain in the study, regardless of any change in circumstances.
a. True
*b. False
to Educational
Instructor Resource Lochmiller, An Introduction
Research SAGE Publications, 2017.
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: Inviting Participants’ Consent and Assent
Question Type: TF
39 Depending on the age and ability of students a researcher might invite verbal assent rather than written.
*a. True
b. False
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: Inviting Participants’ Consent and Assent
Question Type: TF
40 When conducting a review of the literature on your research topic, it is not necessary to obtain approval from the Institutional Review Board.
*a. True
b. False
Learning Objective: 4-3
Cognitive Domain: Comprehension
Answer Location: Legal and Institutional Ethical Requirements
Question Type: TF
41 Institutional Review Board requirements and protocols are exactly the same across all institutions.
a. True
*b. False
Learning Objective: 4-3
Cognitive Domain: Comprehension
Answer Location: Legal and Institutional Ethical Requirements
Question Type: TF
42 When a researcher guarantees anonymity for research participants, he/she will still have knowledge of some aspect of participants’ identities.
a. True
*b. False
Learning Objective: 4-5
Cognitive Domain: Comprehension
Answer Location: The Role of Ethics in Carrying out a Study
Question Type: TF
43 To avoid any dilemmas around authorship, it is important to have conversations with your research colleagues early in the research process.
*a. True
b. False
Learning Objective: 4-7
Cognitive Domain: Knowledge
Answer Location: The Role of Ethics in Reporting Research Findings
Question Type: TF
44. A colleague who was not actively involved in collecting data and writing up the findings for a research project should not be given first authorship for the research report.
*a. True
b. False
Learning Objective: 4-7
Cognitive Domain: Comprehension
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Answer Location: The Role of Ethics in Reporting Research Findings
Question Type: TF
45. The role of ethics in the research process is primarily to ensure the researcher produces a highquality research report.
a. True
*b. False
Learning Objective: 4-1
Cognitive Domain: Comprehension
Answer Location: Ethics and Educational Research
Question Type: TF
46 A conflict of interest occurs when a researcher will not benefit financially from the research that he or she is conducting.
a. True
*b. False
Learning Objective: 4-5
Cognitive Domain: Knowledge
Answer Location: Ethics and Educational Research
Question Type: TF
Essay
Type: E
47 What are three potential factors that will determine how you, as a researcher, should invite consent from your potential research participants? Please describe how each of these will determine how you go about obtaining consent.
*a. Answers may vary. Possible factors include: age of participants (may need to ask for consent from parents/guardians in the case of participants under 18), capacity of participants (how you communicate about research so that participants understand processes and risks), whether participants are a vulnerable population (caution so as to avoid miscommunication around benefits of research), IRB guidelines for your particular institution.
Learning Objective: 4-2, 4-5
Cognitive Domain: Application
Answer Location: Inviting Participants’ Consent and Assent
Question Type: ESS
Type: E
48. You plan to engage in a research study with several collaborators at different institutions. List three ethical issues that you would need to consider as you conduct your research study.
*a. Answers may vary. Possible issues include: securing data for use by more than one researcher, ensuring participant confidentiality, meeting all IRB requirements for different institutions, determining authorship order for final research report.
Learning Objective: 4-1, 4-5
Cognitive Domain: Application
Answer Location: Considering Ethics Across the Research Process
Question Type: ESS
Type: E
49 You have decided to conduct research that includes focus groups with teachers, which you will transcribe and analyze. Regarding your data, what are three steps you should take to help protect your participants’ privacy?
*a. Answers may vary. Possible steps include: securing data files on a personal computer that is password-protected, assigning pseudonyms to participants in transcripts, removing identifying information from transcripts
Instructor Resource
Lochmiller, An Introduction to Educational Research
SAGE Publications, 2017.
Learning Objective: 4-6, 4-7
Cognitive Domain: Application
Answer Location: The Role of Ethics in Carrying Out a Study
Question Type: ESS
Type: E
50 In addition to obtaining IRB approval from your institution, what are three ethical considerations necessary for conducting research in a school or school district?
*a. Answers may vary. Possible considerations include: whether the school/district has an additional IRB requirement, approval from central office/school administrators, how to gain access to classrooms/schools/district of interest
Learning Objective: 4-4
Cognitive Domain: Analysis
Answer Location: Managing Research Relationships with Schools and Districts
Question Type: ESS
Type: E
51 As you plan to embark on a research study, what are three primary ethical issues that should be considered?
*a. Answers may vary. Possible issues include: whether the proposed research topic is ethical, disclosure of any conflicts of interest, ethical considerations for particular research design, inviting participants, procedures for protecting participants’ privacy
Learning Objective: 4-5
Cognitive Domain: Application
Answer Location: The Role of Ethics in Designing and Planning a Study
Question Type: ESS
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The discovery of ataxic phenomena as a symptom of peripheral neuritis has added another to the long list of pseudo-tabic affections. Indeed, Dejerine, who greatly advanced our knowledge of this affection, undertook on the strength of his discovery to place tabes among the peripheral affections complicated by secondary affection of the cord. In a large number of cases of peripheral neuritis, particularly the alcoholic form described by Fischer, the static ataxia, belt sensation, bladder trouble, and reflex iridoplegia are absent. In tabes the severe pains, if influenced by deep pressure at all, are affected favorably, but in peripheral neuritis pressure on the affected nerve-trunk greatly aggravates the trouble.
In addition, those severe forms of neuritis that lead to ataxia, abolition of deep and other reflexes, are accompanied by qualitative electrical changes and atrophic paralysis—features not found in tabes. Sometimes a disseminated neuritis will become so generalized as to cause diplopia and other evidences of ocular paralysis through the affection of the peripheral nerves, but, so far as my observations extend, not with the characteristic pupillary phenomena of tabes.
Cerebellar disease, alcoholic and hysterical neuroses, sometimes produce ataxia, and this may be associated with one or more of the other characteristic symptoms of tabes. Cerebellar ataxia is usually very different from that of tabes, as far as the locomotor element is concerned. In tabes it is the movements the patient makes which cause him to stagger; in cerebellar disease those movements made to prevent staggering are usually co-ordinated. The gait properly called cerebellar is therefore very different from that of tabes. There is, however, static ataxia in both cerebellar and posterior-column disease. In addition, the knee-jerk may be abolished in the former, heightening the resemblance: the history of the case is, however, decisive where these latter symptoms might lead to doubt. Bladder trouble in cerebellar disease is not an early feature, nor are paræsthesias and delayed pain-conduction intrinsic features, of cerebellar disease. Their presence is in favor of tabes.
Alcoholic ataxia is very rapid in its course, while tabic ataxia is exceedingly slow. In addition, the former is accompanied by atrophic paralyses as early features; if such occur in tabes, they occur late in the disease, and are not marked by degenerative electrical reactions as alcoholic ataxia is. This disorder is usually, too, accompanied by fever, which is an exceptional, and certainly never an intrinsic, feature in tabes. Discontinuing the alcoholic poison is usually followed by rapid amelioration of the ataxia. Hysterical ataxia exceptionally apes the ataxia of tabes; the presence of other hysterical phenomena, the rapid and bizarre change of the symptoms, are distinguishing features. There are cases of hysterical ataxia accompanied by concentric limitation of the field of vision.84 The outline of the limitation is strikingly like that accompanying atrophy, but repeated examination shows a variation of a kind not found in the latter. The blind field advances in one direction to recede in another on one day, and reoccupies the latter and recedes from the former on the next.
84 Landesberg, Journal of Nervous and Mental Diseases, vol. xiii. 2.
Ataxia after most acute diseases, such as scarlatina and typhoid, is not usually accompanied by loss of the knee-jerk, and never by reflex iridoplegia. The ataxia after diphtheria is complicated by the former, but, as already stated, the very opposite state of the pupil serves to distinguish it.
The hereditary form of tabes is frequently confounded to this day with true tabes dorsalis. The family history and age of the patient are collateral evidences of the nature of the case. In the hereditary form the sphincters, optic nerves, and the eye-muscles are not affected; in the acquired form the first and last are always involved; and this involvement occurs so early in the disease as to constitute a valuable discriminating feature. Although the locomotor ataxia is very similar in the two affections, static ataxia, the symptom manifested on closing the eyes, is not found in the hereditary form, while it is found more constantly than even the locomotor ataxia in the acquired form.
The electrical reactions in tabes are either normal or quantitatively slightly increased. This serves to distinguish it from all spinal affections accompanied by marked paralysis. In the later stages, when some atrophy results, there may be a quantitative decrease, but these are without qualitative changes. A change may occur in those exceptional cases where the anterior gray horns become involved, but we then have to deal with a true complication; and complications can be recognized only in their development and by the application of the diagnostic criteria characterizing the complicating disorder when of an independent occurrence.85
85 It is not impossible that many of the symptoms described as occurring in advanced tabes are due to independent focal disease. In the case of a female aged seventy years mentioned by Hirt (Berliner klinische Wochenschrift, 1885, No. 26), who developed hemiatrophy of the tongue, probably from a hemorrhage in the brain isthmus, there was no tabes, although this symptom when found with tabes is supposed to be part of the tabic symptom-group.
The TREATMENT of tabes dorsalis will be considered at the end of this article, together with that of the sclerotic processes generally. The duration and prospects of the disorder have been considered with the clinical history. In the vast majority of cases the course of the illness is progressive. A few cases have been reported, but in the only instance where an autopsy was made to test the matter it was found that the sclerosis had not been removed. It may be therefore stated that an arrest of the disease is possible, but that restitution of the nerve-elements, once destroyed, is impossible.
Spastic Spinal Paralysis.
SYNONYMS.—Primary lateral sclerosis. Tetanoid pseudo-paraplegia, (Seguin), Tabes dorsal spasmodique. This symptom-group was first recognized by the American neurologist Seguin86 thirteen years ago; two years later Erb87 described it under the title at the head of this section, and about the same time Charcot88 attributed the well-
marked clinical signs thus discovered on both sides of the Atlantic to a primary sclerosis of the lateral columns of the cord in that portion which is known as the crossed-pyramid tract, and whose isolated disease had been known to Türck,89 but not clearly brought into relation with what is now regarded as one of the most remarkable and recognizable of spinal symptom-groups. Our earlier knowledge of the disease has been much confused through the discovery by Charcot90 of the same lesion to which spastic paralysis is attributed by him, in a female who had been suffering from permanent contractures of all extremities, and which he persisted in regarding as hysterical. The only solution of the difficulty is to accept one of two alternatives—either believing that the sclerosis was in this case primary, in which case contracture cannot be regarded as hysterical, or that it was secondary to protracted disuse of the limbs, in which case it proves nothing. An additional source of confusion has been the discovery of an analogous affection, now regarded as clinically and pathologically distinct, known as amyotrophic lateral sclerosis, and of a corresponding affection of the lateral columns in a large number of cases of paretic dementia.
86 E. C. Seguin, “Description of a Peculiar Paraplegiform Affection (tetanoid pseudoparaplegia),” Archives of Scientific and Practical Medicine, February, 1873. Erb's statement (Ziemssen's Cyclopædia, vol. xi. 2, p. 628) that Seguin's description includes symptoms which do not properly appertain here can no longer be sustained, in view of the similar and identical symptoms which have been since attributed to the same affection and to amyotrophic lateral sclerosis by later writers. There seems to be no question that the priority of recognition of the clinical form belongs to this side of the Atlantic.
That Seguin's title was not as badly chosen as Erb implies in his first reference to it may be inferred from the fact that Strümpell, without any reference to Seguin, and evidently independently of him, uses the following language ten years later: “As also in these cases, the movements are not immaterially influenced by the ever-occurring spasms, a motor disturbance may be simulated which we feel inclined to term spastic pseudo-paralysis, or, more correctly, pseudo-paresis” (Pathologie und Therapie).
87 “Ueber einem wenig bekannten spinalen Symptomencomplex,” Berliner klinische Wochenschrift, 1875, No. 26.
88 “Sclérose primitive de la partie postérieure des cordons antero-lateraux,” Gazette médicale de Paris, 1874, pp. 38, 39.
89 “Ueber primäre degeneration einzelner Rückenmarkstränge,” Sitzungsberichte der Kaiserlichen Akademie der Wissenschaften zu Wien, MathematischNaturwissenschäftliche Klasse, Band xxi. Jahrgang 1856, p. 112.
90 Gazette hébdomadaire, 1865, 7.
CLINICAL HISTORY.—Spastic paralysis manifests itself chiefly in three symptoms: first and most noticeable, a condition of rigidity of the limbs; second, an increase of all the muscular reflexes; and third, a true motor paresis. It manifests itself in the adolescent or middle period of life, usually without any noticeable cause, beginning in the lower extremities, and, if progressive, involving the muscles of the trunk and arms. The invasion of the disease is first marked by an increase in the excursiveness of the muscular phenomena, such as the knee-jerk and the ankle-clonus. But while this reflex irritability is originally only manifested when the diagnostician applies his special tests, it soon becomes so great that the most trivial influence suffices to bring about exaggerated muscular reaction. The mere tension of a tendon in walking, the weight of the limb, the slightest change in position, suffice to produce reflex muscular tension. The muscles feel firm and stiff to the touch, as if permanently contracted, and the lower limbs, as a result of the combined contraction of special muscular groups, assume a characteristic position—namely, an extension contracture of the leg on the thigh and a flexion of the foot on the leg. In advanced cases the contracture is so extreme that it is almost or actually impossible to flex the leg and to extend (dorsiflect) the feet. This is particularly noticeable when it is attempted to overcome the strained position suddenly, while gradual traction will often succeed in relaxing the contracture. The latter procedure succeeds because sudden traction of the tendons and the reflex contraction thereby provoked are avoided by it. Gowers compares this symptom to the mechanism of a clasp-knife. When the leg is
slowly extended it yields to the manipulator's influence, but as soon as it reaches full extension it remains like the blade of the knife fixed by a spring.
Although some degree of muscular weakness is experienced in the beginning of the affection—often more marked in one leg than the other—it is insufficient to account for the grave and characteristic disturbance of locomotion. This is due rather to the stiffness of the limb resulting from the morbid muscular spasm. As the limbs are rigid, the steps are short; the leg not being flexed, and consequently not being lifted from the ground, the gait consists in an awkward shuffle.91 The feet are in continuous contact with the ground, and it is observed that there is a tendency to walk on tiptoe, owing to the contracture of the gastrocnemius, in this respect resembling the pesequinus position. In some cases it has been observed that the reflex excitability was so great that the mere need of urinating brought on a sudden tonic spasm, and there have been noted others in which the very first spasmodic phenomena occurring in the history of the case appeared while the patient emptied his bladder.92
91 It was Theodore Simon, I believe, who first suggested the registration of peculiarities in gait by using sand on the floor or compelling the patient to walk over large sheets of paper with black-leaded shoes. Among the varieties of gait noted in paretic dementia by him he accurately describes that of spastic paralysis (Die Gehirnerweichung der Irren, Hamburg, 1871).
92 Westphal, Archiv für Psychiatrie, xv. p. 224; ibid., p. 229.
When the upper extremities are involved, the same initial muscular weakness and exaggerated reflex excitability are noted, but the contracture at the elbow, unlike that at the knee, is usually in the flexed position.
If from any cause the spastic phenomena happen to be in the background temporarily, so as to admit testing the voluntary muscular power, it is always found to be grossly impaired.93
93 This statement is made by Westphal, but as he considers the only pure cases of pure primary lateral sclerosis to be those associated with paretic dementia, and it is in the latter form alone that there are marked exacerbations and remissions in the spastic symptoms, it probably does not apply to uncomplicated spastic paralysis.
As the disease progresses locomotion becomes impossible; the advancing rigidity of the trunk-muscles renders sitting impossible, and the patients become bedridden. But even in this advanced stage no sensory or vegetative disturbances were noted. The cutaneous reflexes sometimes appear exaggerated, but this is not an evidence of sensory hyperexcitability, but of the increased ease with which the motor response is elicited—a feature which is also illustrated by the spasm resulting from fulness of the bladder or even from the mere act of micturition. Ataxia has never been noted in pure cases, and the uncertainty in gait sometimes noted at the onset of the disease is secondary to the motor weakness and the interference with free mobility by the, as it were, frozen state of the muscles. Occasionally the paresis becomes a veritable paralysis, but this occurrence is limited to one or several muscular groups. Cases are related in which the spastic symptoms occurred on one side, involving one arm and leg for years before involving the other. It is not improbable that these were cases of some obscure cerebral affection. Other exceptional cases in which the spastic phenomena appeared first in the arms are better authenticated.
The early occurrence of ankle-clonus in this disease heralds the appearance of apparently spontaneous clonus when the toes are put on the ground, and later on of cramps of the gastrocnemius or other muscles, which produce an exacerbation of the existing stiffness. Occasionally sudden spasms occur while the patient is at rest, and which resemble the sudden shocks which healthy persons occasionally experience when about to fall asleep.
The electrical reactions show little that can be called characteristic, and there is little unanimity among observers on this point. The majority agree that there is a slight quantitative decrease of both faradic and galvanic excitability in parallelism with the degree of
paresis. In the pure form of the disease there are no other symptoms than those mentioned. Should evidences of involvement of the gray substance of the cord or the cranial nerves be added, it is a certain indication that we have to do with the similar but far from identical affection, amyotrophic lateral sclerosis.
The course of the disease is extremely slow and its development insidious. It is considered incurable, and although a few cases have been described as terminating in recovery, the most recent and reliable annals fail to make mention of any cure in a well-established case of spastic paralysis. The disease is not in itself fatal, death usually occurring from intercurrent affections.
ETIOLOGY.—Our knowledge of the causes of this disease is practically nil. It has been, like tabes dorsalis, attributed to a family tendency Excessive sexual indulgence, over-exertion, and syphilis have been recorded as possible causes in the few cases in which an etiological assignment could be attempted. Tuczek surmises that the spastic phenomena of lathyrism, a constitutional disease analogous to pellagra and ergotism which is observed in those who live on bread prepared from a legumen grown in Italy (lathyrus bean), and which manifests itself in spastic symptoms, may be due to an affection of the pyramid tract, just as tabes ergotica is due to an affection of the posterior columns. One case of spastic paralysis of a severe nature, associated with scanning speech, in consequence of a lightning-stroke, is reported by Demme.94 I have been able to assign a cause in but two cases of spastic paralysis. Both were in Quakers; in both there had been a history of almost incredible sexual excesses. The disease in both involved the muscles of the jaw and face. An autopsy in the one case revealed no lesion whatever.95
94 “Bericht über die Thätigkeit des Jennerischen Kinderspitals,” Wiener medizinischer Blätter, 1884, No. 23.
95 The contracture in the lower extremities, differing from the rule, was a flexion contracture.
MORBID ANATOMY AND PHYSIOLOGY.—Although Charcot's announcement that spastic paralysis is due to sclerosis of the crossed-pyramid tract was made with great positiveness, the more careful authorities have not committed themselves to his view without reservation. Their reserved position is the result of some observations which certainly show that there is no constancy between the distribution of the lesion and the distribution of the spastic paralysis;96 while, on the other hand, characteristic spastic symptoms have been noted with purely cerebral lesions.97 Morgan and Dreschfeld98 publish cases in which the lesion was stated to be characteristic, but as the cell-groups in the anterior horn were found by them to have been more or less affected, it is evident they had cases of amyotrophic lateral sclerosis to deal with. In view of similar revelations in a large number of the cases that had been considered as spastic paralyses during life, and in which similar findings were found after death, Westphal,99 one of the most critical students of the subject, concludes that thus far an anatomical basis has not been demonstrated with any constancy for the cases of spastic paralysis uncomplicated with paretic dementia. With this disease a sclerosis of the lateral column, apparently independent of the cerebral affection, is often found. It has no continuity, as a rule, with the cerebral lesion, and it may be limited to special districts of the cord. It is not usually intense enough to produce material destruction of the tract itself, and for this reason, probably, we do not find any other symptoms than a paretic weakness and an increase of the patellar and other muscular phenomena developed in the majority of paretics. In some, however, the characteristic spastic gait and muscular rigidity do develop. Westphal conjectures that if paretic dements lived as long as the sufferers from uncomplicated spastic paralysis, they would ultimately show the typical symptoms.100 Numerous observations, however, show that the presence and intensity of the spastic symptoms in paretic dementia are not related to the presence and intensity of lateral-column lesion. Thus, Zacher101 failed to find such lesion in a case where the spastic symptoms had been well marked. It must be remembered, in drawing conclusions regarding the pathogeny of simple spastic paralysis from the lateral-cord affection and associated symptoms of paretic dementia, that the possibility of the
lesion of the pyramid tract in this affection being secondary to disuse102 cannot be excluded. On the other hand, the symptoms of most paretic dements presenting lateral-column lesion differ in some respects from those of a pure spastic paralytic. There is a precedent clumsiness and helplessness of movement; the patient stumbles and trips more than is the case with the pure spastic gait; he wavers after suddenly turning around, and there is considerable tremor with intended movement. There is also more exacerbation and remission of these symptoms than is the case with true spastic paralysis, and it is observed that the exacerbations usually follow apoplectiform and epileptiform attacks, thus showing that the cerebral condition, after all, may be the determining factor.
96 Fischer and Schultze (Archiv für Psychiatrie, xi. 3) report an impure case in which, with exquisite spastic symptoms in the neck and arms, the degeneration of the pyramid tract was limited to the dorsal part of the cord.
97 Schulz (Deutsches Archiv für klinische Medizin, Band xxiii.) and Strümpell (Archiv für Psychiatrie, x.).
98 Journal of Anatomy and Physiology, xv. p. 510.
99 Archiv für Psychiatrie, xv. p. 246.
100 Another feature which antagonizes the development of spastic phenomena in paretic dementia is the lesion of the posterior columns which often ensues. In proportion as this is developed it neutralizes the exaggerated knee-jerk, stiffness, and spastic gait.
101 Archiv für Psychiatrie, xiii. p. 155.
102 It has been claimed against this view that if this were so the degeneration of the pyramid tract should be accompanied by degeneration of the cells in the anterior horn. This claim assumes that the cells and the pyramid tract are directly continuous, but the most modern researches, those of Von Monakow, confirming an older observation of Homén's, show that a system of small cells in the lateral reticular processes is interpolated; which I can confirm. It is certainly compatible with an atrophy from disuse of the voluntary tract that the cells themselves, presiding over
reflex and nutritive functions not necessarily disturbed in paretic dementia, should remain nearly intact.
There is another respect in which the two conditions differ. Spastic paralysis is usually an ascending affection, the lower extremities being involved first, the trunk next, and the upper extremities last. But in paretic dementia the spastic phenomena develop in both upper and lower extremities simultaneously, and there are universal tremors, probably of irritative origin. The lower extremities are not commonly in the extension contracture of spastic paralysis, but in the same flexed position as the arms, the adductors usually preceding the flexors in becoming rigid. If it be added to this that the lateralcolumn lesion in paretic dementia appears to answer all the requirements which could be made of the lesion on theoretical grounds were the case one of pure spastic paralysis—that is, that its area decreases upward—the inconsistency of the observed anatomical and clinical facts becomes strongly evident. Thus far, the attributing of spastic paralysis to a primary lesion of the pyramid tract rests in a few contested cases, on a number of doubtful analogies, and on the undoubted fact that sclerosis of this tract in the event of a myelitis is followed by pronounced spastic symptoms in all those muscles which derive their voluntary innervation from the part of the tract which lies below the level of the lesion.
The situation of this tract, which was not discovered by Türck and Flechsig, as is usually supposed, but accurately known to Burdach103 in 1819, may be roughly stated as follows: It lies in the dorsal half of the lateral column, making up the bulk and core of this part of the column. It is separated from the pia mater by the direct cerebellar tract, and from the posterior gray horn by a narrow zone of fibres differentiated by Lissauer (see Tabes). It is connected with the lateral reticular processes, and in its cephalo-caudal course becomes gradually attenuated, giving off its fibres to these processes, thus to be exhausted in the lower part of the lumbar enlargement of the cord, where it approaches, if it does not actually reach, the surface. The fibres controlling the voluntary motions of the lower limbs, and which have a longer course to run before they reach the brain than
those which mediate the voluntary control of the arms, are situated nearest the lateral boundary of the cord. Where the spastic phenomena are mainly marked in the lower limbs the sclerotic process has been found most marked in the corresponding area.
103 Vom Bau und Leben des Gehirns. This gifted author says that the crossedpyramid tract lies in the lateral column of the cord, behind a line corresponding to the attachment of the ligamenta denticulata and removed from the surface.
The progress of this affection has not been materially modified in any case by treatment. The same measures employed in sclerotic processes generally, particularly galvanism and warm baths, are recommended. It is difficult to understand what good effect ergotin, which is mentioned by a number of the German writers, can have in a disease of this nature.
Amyotrophic Lateral Sclerosis.
Amyotrophic lateral sclerosis, so named by Charcot104 and Joffroy, who first described it, consists in a disease affecting both the conducting tracts and nuclear centres of the motor system of the spinal cord and medulla oblongata, manifesting itself in a combination of atrophic and irritative phenomena on the part of the muscles. The relations between the symptoms and lesions of this disease rival in constancy and preciseness those noted in typical tabes dorsalis. As the variations in the mode of invasion and distribution of the lesion account for the widely-differing clinical types of the disease, and the advanced state of our anatomical and physiological knowledge of the cord enables us to interpret the reason of this difference, we shall invert the usual order and discuss the morbid anatomy first.
104 In 1869 and 1874 (Leçons sur les Maladies de la Système nerveux) Charcot termed this, and one other form of disease associated with muscular atrophy, amyotrophic, to distinguish them from the myopathic forms. He considers amyotrophic
lateral sclerosis as deuteropathic, the nuclear atrophy being secondary to the lesion of the white substance, and the progressive muscular atrophy of the type described by Duchenne and Aran as a protopathic form of the amyotrophies. Among the true myopathies he enumerates pseudo-hypertrophic paralysis, Erb's juvenile form, Duchenne's infantile form, certain mixed forms, and, without justification, Leyden's hereditary forms (report by Marie et Guivron, Progrès médicale, 1885, No. 10).
MORBID ANATOMY.—In advanced cases of amyotrophic lateral sclerosis there is found marked sclerotic degeneration of both the crossed and the uncrossed pyramid tracts; atrophy of the cells of the anterior horns; atrophy of some of the nuclei of the motor and mixed cranial nerve, particularly the hypoglossal and spinal accessory; atrophy of the anterior roots of the spinal and the roots of certain motor cranial nerves; and, finally, atrophy of the voluntary muscles. The greater part of the tract through which the voluntary impulse travels after leaving the voluntary motor-fields of the cortex is therefore continuously involved; and it would seem that there are cases (Kahler-Pick's) where the entire motor system is affected, the morbid process demarcating the course taken by the motor impulse through the cerebro-spinal fibre-labyrinth from the cortical motor-field down to the muscles.
The morbid process in the muscles consists of a narrowing of the fibres, which subsequently lose their transverse striation and undergo a granular disintegration. Sometimes a muscle disappears entirely; usually the connective-tissue elements, including the interstitial cellular and adipose tissue, undergo proliferation, so as to mask the wasting of the muscles to some extent.
The morbid process in the nervous system is also a simple degenerative process. The nerve-fibres and cells atrophy first, and the connective-tissue proliferation which marks the sclerotic change of the diseased area is a secondary process. There is still considerable dispute among authorities as to which segment of the motor-conduits the degeneration begins in. But from the great difference found in the individual cases which have become the subject of patho-anatomical studies there can be little doubt that
there is no uniformity in this respect. In some cases the lesion is far advanced in the lateral columns, while the anterior horn is but slightly involved; in others the reverse is found. Sometimes the nuclei of the motor cranial nerves are the chief foci of disease; at others they are the least affected parts of the motor apparatus. As we shall see, there are differences in the clinical picture corresponding to the variations of the anatomical findings.
CLINICAL HISTORY.—In typical cases the first symptom is a tired feeling in one arm or leg, usually the former; in dextral persons the right arm, the one which is subject to the severest strain, is most frequently the first to be affected. With the increase in this tired feeling there develops actual loss of power; the muscles become wasted, and the other arm becomes involved. Often it is observed that the right leg suffers with the corresponding arm, but as a rule the lower extremities do not become involved to any marked degree within the first six months of the illness. It is then noted that a gait not unlike that of spastic paralysis is noted, but with more loss of motor power and less stiffness. There is, however, this noteworthy difference between the affection of the lower and that of the upper extremities: that atrophy and loss of power are more marked in the latter, and spastic phenomena in the former. The knee-jerk and other deep reflexes are greatly increased, and ankle-clonus is usually very well marked. As with spastic paralysis, there are no visceral or sensory disturbances. Unlike that affection, there are qualitative changes in the electrical reaction of the muscles105 in amyotrophic lateral sclerosis. The degeneration reaction is found in the atrophied divisions, and particularly in such groups as those of the thenar and hypothenar eminence, which undergo complete atrophy at a comparatively early period of the disease.
105 Moeli, Strümpell, Pick, and Mierzejewski describe cases in which only quantitative changes were found. They were such in which spastic phenomena preponderated at the time of the examination.
Soon after the spastic and atrophic involvements of the lower extremities, symptoms indicating the involvement of the cranial
nerve-nuclei are developed, usually after the disease has lasted a year or so. Deglutition becomes difficult and speech indistinct, the general picture of a glosso-labio-laryngeal paralysis being imitated. The patient cannot pucker his lips, his lingual muscles undergo atrophy, and fibrillary and fascicular twitches are noted in the tongue and lips. But just as the atrophic affection of the muscles of the arms and legs differs from that of progressive muscular atrophy in the fact that the deep reflexes are exaggerated with amyotrophic sclerosis, so in the bulbar symptoms of the latter it is found, unlike the typical form of bulbar paralysis, that the jaw reflex is increased.
The duration of the disease may be stated at about three years, death usually occurring in consequence of the involvement of the cranial nerves. There are cases recorded where the disease was almost simultaneously developed in all four extremities and the tongue, reaching a high degree within a year (Mierzejewski). It is generally agreed that the reason contractures do not develop in typical cases of this kind, although the lateral column is sclerosed and spastic phenomena occur early in the disease, is the destruction of the cell-groups in the anterior horn. The reflex arch through which a reflex contracture would be mediated is broken, or rather weakened, in that part of its course which passes through these cells, and therefore a contracture is as efficiently antagonized as it would be if the posterior roots were divided. Still, in some cases a frozen attitude of the lower extremities is very well marked (Vierordt, Zacher). To reconcile these conflicting observations it has been suggested that it may be regarded as a question of speed between the progress of the pyramid-tract and the anterior-horn lesions. If the former be much in advance of the latter, spastic phenomena will preponderate and contractures be possible, to disappear with the subsequent anterior-horn lesion. But if the latter precede and preponderate, the spastic phenomena will be in the background and contractures impossible. Indeed, Zacher106 suggests that there may be an ascending form in which the lesion of the pyramid tract is secondary to the nuclear atrophy, corresponding to the typical class of cases on which Charcot based his first description of the disease, and a descending form in which the pyramid tract is first affected and
the nuclear cell-groups follow Vierordt107 and Kahler108 express similar opinions. The latter goes so far as to suggest that progressive muscular atrophy, progressive glosso-labio-laryngeal paralysis, and amyotrophic lateral sclerosis are really due to one and the same kind of degenerative process, merely differing in location. There is certainly, as he claims, a remarkably complete chain of cases, beginning with such (1) in which spastic paralysis preponderates, passing thence to (2) those in which some muscular atrophy preponderates, then (3) those in which muscular atrophy is in the foreground and the spastic phenomena are slight, and ending with (4) the pure atrophies. A similar transition may be established on the regional principle between pure glosso-labio-laryngeal paralysis and amyotrophic lateral sclerosis, for there are cases of this affection in which the oblongata symptoms preponderate throughout, and the focus of the disease is formed there, just enough lesion being demonstrable in the pyramid tract and the spinal gray matter to prove the family relationship of what clinically appears as a spastic bulbar paralysis.109
106 Archiv für Psychiatrie, xv. p. 416.
107 Ibid., xiv. p. 397.
108 Zeitschrift für Heilkunde, 1884, p. 109.
109 Such a case is described by Freund in Deutsches Archiv für klinische Medizin, xxxvii. p. 405.
PROGNOSIS.—As far as the typical cases of this disease have been studied, a fatal termination seems to be invariable. Seeligmüller reports a few cases in which the progress appeared to become arrested, but it is not clear that these were not in reality cases of some juvenile form of muscular atrophy. Not only is the affection in adults fatal, but it is so in a short period of time as compared with other spinal disorders, and particularly with the related disorder spastic paralysis. Few patients survive the third year of their illness; a number do not live to that length.
The Combined Forms of Sclerosis.
A number of cases of chronic sclerotic disease of the cord have been discovered and analyzed during the past decade, which, while they show the regularity of distribution noted in posterior and lateral scleroses, differ from them in involving at once more than one column of the spinal cord. Usually, it is the posterior column and the posterior part of the lateral column which are affected. The symptoms constitute a combination of those of tabes dorsalis and of spastic paralysis. But this combination does not represent a mere addition of symptoms; where the tendency of the two diseases conflicts, they neutralize each other. Thus the tendency of lateral sclerosis uncomplicated by posterior sclerosis is to increase the patellar jerk; when posterior sclerosis complicates it, the jerk is annihilated. The degeneration of the lateral column and the ensuing motor paresis in like manner neutralize the ataxic character of the gait by limiting its excursiveness.
The upper extremities are usually involved equally with the lower. In the cases of Kahler-Pick considerable atrophy of the muscles developed; in those of Prévost and Westphal this was not very noticeable. In a few cases, where the posterior sclerosis did not involve the lumbar part of the cord, spastic symptoms were noted in the lower extremity. In the only case of combined sclerosis now under my observation this peculiarity, noticed by Prévost, is well marked. In one of Westphal's cases there was evident mimic ataxia. The few cases of this affection observed show so many variations that it would be at present premature to attempt sketching a common clinical type. The majority of the subjects were affected between the twenty-fifth and forty-fifth years.
ETIOLOGY.—Little is known of the causes of this disease. Usually beginning insidiously, no special mode of origin can be determined. Surface chilling has been assigned in a case by Erlitzky and Rybalkin, and others are reported to have begun during pregnancy
One of Westphal's cases developed in a lithographer who had been subject to epileptoid fits.
DIAGNOSIS.—As yet we have no reliable criteria for distinguishing between a combined sclerosis and certain forms of diffused sclerosis during life. Ballet and Minor110 found such a sclerosis diffusely involving the posterior and lateral columns of the cord in a case where they were justified from the symptoms in expecting a combined fascicular sclerosis of these columns, and after a careful study of all similar cases collated by them in consequence of this experience, they came to the conclusion that a number of affections of different origin, but eventually involving both columns, may impose on the observer as combined fascicular sclerosis during life. Diffuse meningo-myelitis is one of these affections, and is far from uncommon, while true system or combined sclerosis is apparently a very rare disease.
110 Archives de Névrologie, vii. p. 44.
The Family Form of Tabes Dorsalis.
SYNONYMS.—Friedreich's disease, the Family form of locomotor ataxia, Hereditary ataxia, Hereditary tabes.
Friedreich discovered a peculiar form of co-ordinating disturbance in a number of children of the same family, which he brought into relation with a lesion of the posterior columns, and which has been since found by him and by subsequent observers to occur in other cases, always affecting several members of the same family, as in the first case observed by him. The clinical and pathological features, though resembling those of the tabes dorsalis of adults in many respects, are distinct in others, and for this reason it is generally assigned a separate place in classification.
ETIOLOGY.—The disorder is usually manifested in juvenile life, the age of the affected subjects varying from the seventh to the twentyfifth year. The male sex preponderates in the statistics of the affection. Some neuropathic vice can always be found in the patient's immediate ancestry, and the limitation of the disease to families burdened by such a diathesis is exemplified in the fact that the ninety individual cases thus far accurately studied occurred in thirty-six families.111 The disease type of the ancestors of the patients is usually different from that of the latter. Alcoholism in the father is one of the commonest forms, but convulsions, hysteria, and insanity are also frequent features of the family history. In a few cases tabes dorsalis, properly so called, was present in the father. In others there was consanguinity of the father and mother. In Musso's group the parents had been brother and sister, and their mother had been a melancholic dement. Three other grandchildren and six grandchildren by the incestuous marriage developed the family form of tabes. In this family the frequent experience of hereditary transmission was verified—that the neurotic taint skipped the intermediate generations.
111 Raffaele Vizzioli, Giornale di Neuropatologia, 1885.
CLINICAL HISTORY.—Usually the first symptom is ataxia of the lower extremities; occasionally this is preceded by severe frontal headache or by vague rheumatoid pains. The inco-ordination is very similar to that of true tabes dorsalis, but swaying on closing the eyes is not noticed early in the disease, as in the latter affection. The arms soon become involved in the ataxia, but cutaneous sensibility and the muscular sense remain either intact or nearly so—a fact utilized with some success by Erb in polemicizing against the theory of Leyden that the ataxia of tabes is due to imperfect sensation. Later in the disease, usually after a few years, a peculiar speech-disturbance is noticed, which resembles the scanning of disseminated sclerosis. It depends on ataxia of the tongue and lips. This is usually associated with nystagmus. About this time the patient develops a different set of motor symptoms from those characterizing the onset of the disease; contractures, paralysis, and atrophy are found in the
affected extremities; sometimes the patients cannot ascend a stair, owing to their inability to lift the feet high enough. Pes equino-varus, deformity of other joints and of the vertebral column, have been observed112 to result from the associated effects of paralysis and contracture. At this stage some sensory disturbance may be developed, formication having been observed toward the close of the history in a number of cases. But the distribution of this disturbance is usually different from that of tabes dorsalis, being more intense in the trunk than in the extremities or evenly marked in the entire periphery
112 H. E. Smith, Boston Medical and Surgical Journal, 1885, vol. cxiii. p. 361.
COURSE AND PROGNOSIS.—The progress of this disease is slow. It has not yet been known to be arrested by any therapeutical procedure. Death rarely occurs directly from the disease by exhaustion; more commonly life is cut short by some intercurrent affection. Unless this occurs the patients may survive the commencement of the illness from eight to forty and more years.
MORBID ANATOMY.—The sclerosis which is found to be the constant lesion underlying this disease corresponds in every character to a combined sclerosis of the pyramid tracts and the posterior columns. Usually, the crossed-pyramid tract is degenerated in its spinal course, and the uncrossed in the cervical and dorsal part, which, in many subjects at least, is its whole extent. The cerebral part of the pyramid tract is not affected. The nerve-fibres found normally in the gray substance are materially reduced, probably in dependence upon the atrophy of the great nerve-tracts.
The lesion of the posterior columns resembles that of true tabes very closely, particularly in the lumbar part of the cord. It is, however, not probable that it commences in precisely the same distribution, and if cases dying early in the disease be autopsied it will be interesting to see whether the initial sclerosis occupies identical fields—a contingency which is unlikely, owing to the profound difference in the initial symptoms of true tabes and the family form. It is claimed by Schultze that in addition to the pyramid and posterior tracts the