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.
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What’s the difference between ALS AND HIRAYAMA’s disease_.pptx
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2. 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.
3. 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
4. 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.
5. 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.
6. 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
7. 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.
8. 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
9. 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.
