14 years old male, right handed , resident of village Parsoli Chittorgarh,
studying in 8th standard, presented with chief c/o:-
• Neck pain with restricted neck movements since– 1 year
• Progressive weakness of both upper limbs right > left – 6 months
• Difficulty in walking with tightness of both lower limbs – 3 months
• Abnormal eye movements from 2 months
• Change in voice and nasal regurgitation of food from 15 days
HOPI
• Patient was apparently well 1 year back when he fell down from bicycle
and after that he developed localized pain at nape of the neck, which
was acute in onset localised in nature and the pain was associated with
restricted movements of neck which was more while looking towards
right side. He has to turn himself to see the objects placed on his right
side.
• For this, he was taken to nearby hospital where pain medication
prescribed with local ointment. Gradually over the span of 2-3 weeks,
pain subsided while restricted neck movements persisted.
• Patient then developed weakness in right upper limb from last 6
months which he first noticed while writing, he found difficulty in
holding the pen, he also had difficulty in buttoning and unbuttoning
his shirt and breaking chapati while eating with his right hand.
Weakness gradually progressed upwards and he started difficulty in
raising his arm above head as whole while pouring the water over
head while bathing and combing hairs.
• Within the span of next 15-20 days, he developed weakness in left
upper limb which progressed in similar fashion.
• He developed stiffness in both lower limbs from last 3 months, he
noticed that he has difficulty in negotiating his right foot into the slipper
and occasionally slippers slip off without his knowledge. Within the span
of next 10-15 days patient started similar complaints in his left lower
limb. It was associated with cotton wool like sensations while walking.
He also had frequent episodes of fall while walking on uneven surface
and this aggravated during night time.
• He developed abnormal up and down movements of both eyes from last 2 months
which he first noticed while washing his face in the morning.
• Since last 15 days his parents noticed change in his voice which became weak with
nasal intonation. He also had nasal regurgitation of liquids and cough while taking
food.
• N/H/O Cough headache/seizures.
• N/H/O blurring of vision.
• N/H/O facial numbness.
• N/H/O tinnitus and hearing loss.
• N/H/O deviation of tongue.
• N/H/O giddiness while turning head or drop attacks.
• N/H/O shortness of breath.
• No history suggestive of horner’s syndrome.
• N/H/O fever/ wt loss/ TB contacts.
• N/H/O bladder and bowel involvement.
• Past history – N/H/O TB, DM.
• Personal history – vegetarian diet, appetite normal, sleep adequate,
normal bowel & bladder habits.
• Family history – not significant.
• Medical history – Used pain medications, no H/O other long term drug
intake
GENERAL EXAMINATION
• Patient averagely built, nourished PR 84/min regular/normovolemic.
• BP 118/72 mm Hg in supine and standing.
• No cyanosis/clubbing/pallor/icterus.
• No neurocutaneous markers present.
• Respiratory system – rate 24/min regular, chest bilateral symmetrical, no added
sound on auscultation. Breath holding time 30 sec, single breath count 18/breath.
• CVS – normal.
• Abdomen – normal.
• Low hair line
• No low set ears/ no high arched palate .
• CNS EXAMINATIION
• HMF– conscious/alert/oriented to time, place and person.
• MMSE – 28/30.
• SPEECH – nasal intonation present but fluency/ comprehension/ repetition intact.
• Cranial nerves
• I – normal
• II - visual acuity 6/6, near vision intact, colour vision normal, no field defect, pupils b/l 3
mm reacting to light. Fundus examination – normal.
• III, IV and VI – no ptosis, extraocular movements normal in all gaze and direction, no
diplopia.
• Nystagmus – present in primary gaze, down-beating, exaggerates on looking downwards.
• V – sensations to pain, temperature and touch intact bilaterally in V1, V2 and V3 segment.
• Corneal reflex – direct and consensual present on both sides.
• Jaw jerk – absent
• No temporal hollowing, no deviation of jaw, muscles of mastication normal bilaterally.
• VII – Normal.
• VIII – Normal
• IX and X- On inspection – uvula deviated to left, with reduced movements
of right palatal arch on telling “Ah”.
• Gag reflex impaired on right side
• XI – decreased shrugging of right shoulder as compared to left with
flattening of shoulder contour on right side.
• XII – tongue central/ symmetrical without wasting and denervation.
• No fasciculations
• Motor examination
• Bulk:- Upper limbs:- right shoulder at lower level than left, flattening of
shoulder contour
Part Rt arm Left arm Rt Left Rt thigh Left thigh Rt leg Left leg
forearm forearm
Circumfer 19cm 19cm 18cm 18cm 35cm 35cm 26cm 26cm
ence
• Tone:-
Tone Right Left
Upper limb Increased (++) Increased (++)
Lower limb Increased (+++) Increased (+++)
power Right left
Shoulder - flexion 4/5 4+/5
extension 4/5 4+/5
adduction 4/5 4+/5
abduction 4/5 4+/5
Elbow - - flexion 4/5 4+/5
extension 4/5 4+/5
Wrist - flexion 4/5 4+/5
Extension 4/5 4+/5
handgrip weak weak
Hip - abduction 4+/5 4+/5
abduction 4+/5 4+/5
- flexion 4+/5 4+/5
extension 4+/5 4+/5
- Knee - flexion 4+/5 4+/5
extension 4+/5 4+/5
Ankle- flexion 4+/5 4+/5
extension 4+/5 4+/5
EHL weak weak
DTR Right LEFT
BICEPS 3+ 3+
SUPINATOR 3+ 3+
TRICEPS 3+ 3+
HOFFMAN POSITIVE POSITIVE
KNEE 3+ 3+
ANKLE 3+ 3+
CLONUS – KNEE ABSENT ABSENT
ANKLE CLONUS PRESENT PRESENT
• SUPERFICIAL REFLEXES – abdominal absent in all 4 quadrants.
• B/L plantar extensor
• SENSORY – loss of pain, touch and temperature below clavicle more on left side
• Joint position sense and vibration impaired B/L in UL & LL.
• Romberg’s positive
• Cerebellar signs – Absent
• No dysmetria/ dyssynergia/ dysdiadochokinesia/ intention
tremors.
• Gait – spastic gait
• Speech – Nasal intonation present
