1. A Case of Amyotrophic Lateral
Sclerosis
- Vandana Valluri

2. 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

3. 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.

4. 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.

5. 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.

6. 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.

7. Drug History
No h/o long 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
 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.

10.  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.

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
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

15. CVS Examination
- Apical impulse 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 breath sounds heard all over the lung on both sides.
No added sounds.

17. GIT Examination
No organomegaly.

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 is a 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

21. Thank You .