What’s the difference between ALS AND HIRAYAMA’s disease_

Understanding Amyotrophic Lateral Sclerosis

● ALS is also referred to as Lou Gehrig’s disease, named after the baseball player who was diagnosed with it. ● Amyotrophic Lateral Sclerosis (ALS) is a nervous system disorder wherein the motor neurons in your brain and spinal cord don’t function properly. The nerve cells break down, thereby reducing functionality in the muscles, voluntary movements, and in some severe cases, breathing too.

Amyotrophic Lateral Sclerosis Symptoms Some of the signs and symptoms include: ● ● ● ● ● ● ● ● ●

Difficulty in performing daily chores or activities Tripping, clumsiness and falling Mood swings and behavioral changes Muscle cramps and twitching in your arms, shoulders and tongue Weakness in your legs, feet, ankles or hands Slurred speech and trouble swallowing Inappropriate crying, laughing or yawning Cognitive and behavioral changes Inability to hold objects and have a proper grip

Understanding Hirayama’s Disease ● Hirayama’s Disease is a non-progressive juvenile spinal muscular atrophy that gradually progresses into the atrophy of the muscles in the arms and forearms. ● Also known as MonoMelic Amyotrophy (MMA), this cervical injury is characterized by muscular weakness and atrophy of distal upper limbs, followed by spontaneous arrest within several years. ● It is known to primarily affect the lower cervical cord, and more often than not, it is diagnosed in men or male adolescents.

Hirayama’s Disease Diagnosis ● Hirayama disease does not have any known causes. ● Clinical observations, however, do show that excessive movement (flexion) of the neck in the upper cervical region causes the membrane covering the spinal cord to become lax. ● This anterior displacement of the spinal cord results in its compression, and thereby, becoming damaged. ● Doctors can diagnose it with EMG and NCS to help distinguish Hirayama’s Disease from other similar disorders.

Hirayama Disease Causes and Symptoms The exact causes of Hirayama’s Disease are not known but certain symptoms are visible, namely: ● ● ● ● ● ● ● ●

Hand muscle weakness and weakened grip Hand muscle wasting Hand muscle cramps Cold hand Clawed hand Twitching of the hand Hand tremors Loss of fine motor control

Differentiating between ALS and Hirayama’s Disease ● Hirayama disease (HD), Amyotrophic Lateral Sclerosis (ALS) and Cervical Spondylotic Amyotrophy (CSA) are all likely to result in atrophy of the intrinsic hand and forearm muscles. However, HD is caused by a benign focal lesion that is limited to the upper limbs. ● Given that ALS is characterized by the degeneration of the upper and lower motor neuronal systems, it is caused by the degeneration of the motor neuron in the brain. Furthermore, ALS is a progressive disease, which means it gets worse over time. As your muscles get weaker, it affects all muscle related movements.

ALS Treatment Stem cell therapy is considered a viable treatment option for ALS, since stem cells can sustain and nurture the diseased motor neurons.

Some activities can prevent future complications: ● Medication can help relieve muscle cramps ● Exercise for Amyotrophic Lateral Sclerosis includes breathing exercises, moderate swimming, walking, bicycling and similar activities that can work on degenerated muscles ● Speech therapy ● Good dietary nutrition

Hirayama Disease Treatment ● It is said that Hirayama Disease cures on its own, with or without any specific treatment, given that it is a self-limited disease. ● Regular physiotherapy can surely help to restore strength in the arm and hand muscles, and prevents joint stiffness and immobility. ● Physical Therapy and Occupational Therapy can play a prominent role in strengthening the muscles and improving fine motor skills ● Stem Cell Therapy is also known to be effective, since stem cells can multiply and can make the copies of other cells. ● Other treatments include wearing a cervical collar that will gradually reduce neck flexion or alternatively, muscle-strengthening physiotherapy exercises. ● Surgical treatments (once confirmed after an MRI) involves an operation of the cervical spine followed by the insertion of a small disc.

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