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A Case of Amyotrophic Lateral Sclerosis
- 1. A Case ofAmyotrophic Lateral Sclerosis - Vandana Valluri
- 2. Bio-data A 46 yearold male patient, who is a worker in a pesticides shop, hailing from Gannavaram, came to the hospital on 24/4/2013 with the Chief Complaints of : - Weakness of both lower limbs from 12 months - Weakness of both upper limbs from 6 months - Hoarseness of voice from 2 months - Difficulty in swallowing from 1 month
- 3. H/o Presenting Illness Thepatient was apparently alright one year ago. 12 months back he first developed weakness in both the lower limbs which was insidious in onset, gradually progressive in nature, spreading from the feet to the knees and then to the hips. 6 months later he noticed weakness of both the hands, followed by difficulty in swallowing, more for liquids, and also difficulty in chewing. The patient also developed hoarseness of voice and slurring of speech since 2 months. He had become dependent for most activities by the end of 10 months. The patients also had episodes of night cramps in the calf muscles. No h/o fever, neck stiffness. No h/o headache. No h/o any vomiting, syncope attacks. No h/o trauma. No h/o numbness, altered sensorium or behavioral abnormalities. No h/o blurred vision.
- 4. Past History No suchcomplaints in the past. The patient is not a known hypertensive or diabetic. No h/o pulmonary tuberculosis, asthma, epilepsy, syphilis.
- 5. Personal History He isa chronic alcoholic(180ml brandy per day) and smoker(40 pack years). Normal bowel and bladder functions, normal appetite. Sleep is disturbed due to nocturnal cramping in the calves.
- 6. Family History The patient’sfather suffered with similar complaints at the age on 42years. He died at the age of 44years due to respiratory insufficiency.
- 7. Drug History No h/olong term drug usage. Allergic History No significant allergic history.
- 8. General Examination - Conscious,coherent, alert. Ill build and ill nourished. - P-pallor present I- C- C- absent L- E- - Vitals : Pulse= 76/min, regular, rhythmic, normal volume, vessel wall condition is normal BP = 120/80 mm Hg RR = 13/min, abdominothoracic Temp – afebrile - Neurocutaneous markers : absent
- 9. CNS Examination Highermental functions: could not be assessed as the patient’s speech is discrete and he is unable to even write due to diminished motor functions of distal muscles of hands.
- 10. Cranial nervesexamination: 1. Olfactory – normal 2. Optic – normal 3. Oculomotor, 4. Trochlear, 6. Abducens – normal 5. Trigeminal – (m)Jaw jerk – brisk; (s) – normal 7. Facial – wrinkling forehead, closing of eyes diminished; clenching of teeth absent; decreased taste perception. 8. Vestibulocochlear – normal 9. Glossopharyngeal – (m)- sluggish palatal reflex, pharyngeal reflex is lost 10. Vagus – gag reflex is diminished 11. Spinal accessory – diminished shrugging of shoulders 12. Hypoglossal – tongue wasting, inability to protrude the tongue, fasciculations of tongue present.
- 11. Motor system: 1.Bulk(nutrition): 2. Tone: Spasticity noticed 3. Power: MRC grading: upper limbs – 3 to 4; lower limbs – 2 to 4 4. Co-ordination: finger-nose test - positive; knee-heel test - positive 5. Involuntary: fasciculations 6. Gait: altered gait rhythm and less steady gait due to foot drop Upper limb Lower limb Right Left Right Left Mid-arm: 9cm 9cm Mid-thigh:
- 12. 7. Reflexes: Superficial:Corneal, conjunctival – normal; Abdominal, cremasteric, plantar – exaggerated. Deep tendon reflexes: triceps supinator knee ankle exaggerated
- 13. Sensory system examination A.Superficial sensations: touch, pain, temperature- present B. Deep sensations: proprioception, vibration- present C. Discriminative sensory function: stereognosis, localisation of touch, 2-point discrimination- present
- 14. Peripheral nervous system Nothickening of nerves. Tinel’s sign negative. Spine and Cranium Meningeal Signs No deformities, height-neck ratio is normal, no tenderness. Kernig’s sign, Brudzinski’s sign and Neck rigidity: absent
- 15. CVS Examination - Apicalimpulse seen in the left 5th intercostal space, half inch medial to midclavicular line; - Apex beat felt - Auscultation: Tricuspid Mitral S1, S2 heard, no murmurs
- 16. RS Examination Vesicular breathsounds heard all over the lung on both sides. No added sounds.
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- 18. Investigations Routine blood investigations,thyroid function tests, collagen vascular profile, protein electrophoresis and immunological profile were normal. Malignancy work-up was negative. Electromyography: active and chronic denervation and reinnervation in all the limbs. Nerve conduction tests were normal.
- 19. Provisional Diagnosis This isa probable case of Amyotrophic lateral sclerosis.
- 20. Differential Diagnosis - Poliomyelitis,Multiple sclerosis - Polymyositis/ Dermatomyositis - Chronic inflammatory demyelinating polyradiculoneuropathy - Brainstem gliomas, central cord tumours - Primary lateral sclerosis - Spinal muscular atrophy
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