Miss.

Mahalakshmi, 10 year old female patient from madurai , studied up to 5th

std, Cooley , Rt handed individual whose reliability of information is fair has come
with

c/o head tilt to left side from 1 yr of age

Frequent falls for 5 yrs

Neck pain for 1 yr

Weakness of left upper limb and lower limb for 3months

HOPI

 Patient was apparently normal at birth, 1 st born female child to non-

consagious marriage, Term delivery, LSCS, attained normal developemental
milestones.
 Patient mother noticed head tilt to left side at her 1 yr of age, patient was
brought to GRH,Madurai ,Paediatric department, where she was reassured
and sent back home.
 Patient was kept on regular follow up, but patient didn’t turn up for follow-
up , no further investiagations were done
 Head tilt was exaggerated during the following years with noticeable head
tilt at her 4 yrs of age
 H/o Frequent falls in the past 5 years, which patients parents ignored due
to her age and playfulness.
 H/o Neck pain, dull aching, localized to neck, with no radiation, aggravated
by movements, not aggrevated by coughing, relieved by medication.
 H/o Weakness in the Left upper limb in the form of difficulty in holding
objects, difficulty in buttoning and unbuttoning shirts for the past 3 months
 H/o Difficulty in reaching for over head objects in left hand, difficulty in
combing using left hand
 Later patient developed, difficulty in holding slippers in left foot, difficulty in
climbing upstairs, H/o bucking of left knee
 Patient able to get up from bed without any difficulty and still now patient
walking without support, doing her day to day work with slight difficulty
 No H/o numbness and decreased sensation of insect bite, hot and cold
sensation while bathing and decreased sensation of clothes
 No H/o cotton wool sensation while walking and unsteadiness while
washing face , electric shock like sensation while extending neck or
difficulty in walking during night.
 No H/o wasting,twitching and fasciculations.
 No h/o cramps ,involuntary movements.
 No h/o decreased sense of smell.
 No h/o diminished vision,defective colour vision.
 No h/o dipopia/ptosis/squint
 No h/o decrease sensation over face/difficulty in chewing.
 No h/o difficulty in frowning forehead/raising eyebrows/puffing of
cheeks/deviation of angle of mouth while smiling /drooling of saliva.
 No H/o hard of hearing/tinnitus.
 No h/o dysphagia/nasal regurgitation/hoarseness of voice/stridor.
 No h/o difficulty in shrugging shoulders or turning head side to side.
 No h/o deviation of tongue while protruding/difficulty in movements of
tongue.
 No h/o tremors , unsteadiness while sitting standing or walking,head
nodding.
 No h/o difficulty in initiating micturition/control the stream of
micturition/fully evacuate the bladder/ feeling of residual urine/increased
frequency of micturition.
 No h/o bowel impaction. No h/o difficulty in initiating defecation /fully
evacuate the bowel.
 No h/o abnormal sweating,palpitations,postural giddiness
 No h/o cough with expectoration/fever.
 No h/o trauma.
 No h/o loss of weight/appetite.
 No h/o dog bite /recent vaccination.
PAST H/O – Not on regular drugs, not a Diabetic, epileptic, asthmatic, no contact
with tuberculosis

Personal h/o – Takes mixed diet

Family h/o – No similar illness in the family

Summary

10 yr old female child presented with insidious onset gradually progressive

neurological illness in the form of head tilt from 1 yr of age, neck pain, localized,
without any radiation, associated with stiff weakness of Left upper and lower limb
without any posterior coloumn , autonomic nervous system, higher mental
functions, cranial nerve, cerbellar involvement

s/o Congential CVJ anamoly with CST involvement (L)

O/E

Patient conscious
oriented
not anaemic
not jaundiced bp-130/80 mm Hg
no cyanosis Lt UL sitting
No clubbing posture
no pedal edema/gen. lymphadenopathy
no neck rigidity/neurocutaneous markers

NO tenderness in cervical spine region

No Low hair line ,

Neck - Height -116 , neck – 9 Ratio – 12.8

Neck Movements – Chin tilt to left side, occiput to right side, Able to flex and
extend her neck without any difficulty, Right Neck rotation – restricted, left – with
difficulty ,Lateral bending – left > right difficulty

Cranial nerve examination

olfactory nerve normal normal

Optic nerve visual acuity 20/30 20/40

Field of vision Normal Normal
Colour vision Normal Normal
Fundus Normal Normal

EOM full EOM full

Oculomotor ,trochlear and abducent nerve No No
ptosis/squin ptosis/squint
t
Ophthalmic Normal Normal
Trigeminal nerve maxillary Normal Normal
Mandibular Normal Normal
Motor Normal Normal
Jaw jerk Absent Absent
Frowning forehead Normal Normal
Facial nerve raising eyebrows Normal Normal
Puffing cheeks Normal Normal
Angle of mouth No deviation No deviation
Rinnes test AC>BC AC>BC
Vestibulocochlear nerve webers test No No
lateralization lateralization
ABC normal normal
Uvula Midline Midline
Glossopharyngeal Palatal movements Normal Normal
nerve and vagus nerve gag reflex Present Present

Shrugging shoulders Normal Normal

Spinal accessory nerve turning head side to side Normal Normal
Trapezius reflex Normal Normal
Hypoglossal nerve tongue movements Normal Normal
Protrusion No deviation No deviation

Examination of spinomotor system

Bulk Right Left

Arm 14 cms 33cms

Forearm 20 cms 20 cms
Thigh 45 cms 45 cms
Leg 28 cms 28 cms

Tone Right Left

Upper limb Increased Increased

Lower limb Increased Increased

Power Right Left

Shoulder flexion 4/5

extension 5/5 4- /5
abduction 4-/5
adduction 4-/5
Elbow flexion 5/5 4-/5
Extension 5/5 4-/5
Wrist flexion 5/5 4/5
Extension 4-/5
Hand grip Good weak
Hip flexion
extension 5/5 4-/5
abduction
adduction
Knee flexion 5/5 4-/5
 Extension 5/5 4-/5
Ankle dorsiflexion 5/5 4/5
Plantar flexion
EHL NO Weakness Weak

Reflexes Right Left

Trapeius Normal Exaggerated
Biceps Normal Exaggerated
Supinator Normal Exaggerated
Triceps Normal Exaggerated
Knee Normal Exaggerated
Ankle Normal Exaggerated
No clonus

Superficial reflex

Corneal Present Present

Conjuctival Present Present
Abdominal Present Absent
Plantar Flexor Extensor

Sensory system

Sensory system Right Left

Pain Normal Normal
Touch Normal Normal
temperature Normal Normal
Joint sense Normal Normal
Vibration sense Normal Normal
Coordination

Finger nose test Normal Normal

Finger finger nose test Normal Normal
Heel knee test Normal normal
 Tandem walking – possible, with difficulty
Rhombergs - negative

Gait and stance –

No involuntary movements .