A Case of Amyotrophic Lateral Sclerosis

- Vandana Valluri

Bio-data

A 46 year old 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

H/o Presenting Illness

The patient 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.

Past History

No such complaints in the past.

The patient is not a known hypertensive or diabetic. No h/o pulmonary tuberculosis, asthma, epilepsy, syphilis.

Personal History

He is a 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.

Family History

The patient’s father suffered with similar complaints at the age on 42years. He died at the age of 44years due to respiratory insufficiency.

Drug History

No h/o long term drug usage.

Allergic History

No significant allergic history.

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

CNS Examination

Higher mental 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.

Cranial nerves examination:

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.

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:

7. Reflexes: Superficial: Corneal, conjunctival – normal; Abdominal, cremasteric, plantar – exaggerated.

Deep tendon reflexes: triceps

supinator

knee

ankle

exaggerated

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

Peripheral nervous system

No thickening 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

CVS Examination

- Apical impulse seen in the left 5th intercostal space, half inch medial to midclavicular line;

- Apex beat felt

- Auscultation:

Tricuspid

MitralS1, S2 heard, no murmurs

RS Examination

Vesicular breath sounds heard all over the lung on both sides.

No added sounds.

GIT Examination

No organomegaly.

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.

Provisional Diagnosis

This is a probable case of Amyotrophic lateral sclerosis.

Differential Diagnosis

- Poliomyelitis, Multiple sclerosis

- Polymyositis/ Dermatomyositis

- Chronic inflammatory demyelinating polyradiculoneuropathy

- Brainstem gliomas, central cord tumours

- Primary lateral sclerosis

- Spinal muscular atrophy

Thank You .