Difference Between ALS and Hirayama's Disease

1
(No Transcript)
2
Understanding Amyotrophic Lateral Sclerosis

• ALS is also referred to as Lou Gehrigs 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 dont 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 Hirayamas Disease

• Hirayamas 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
Hirayamas 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 Hirayamas Disease from other similar
  disorders.

6
Hirayama Disease Causes and Symptoms

• The exact causes of Hirayamas 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 Hirayamas 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.

10

• Thank You