AMYOTROPHIC LATERAL SCLEROSIS PLUS SYNDROME (COIMBATORE MOTOR NEURON DISEASE)

Dr. Moses P. Moorthy, Dr. M. Sacratis, Prof. A. V. Srinivasan

Abstract


CASE REPORT:
26 Year old Male admitted with history of unsteadiness with swaying
while walking for the past two years, which is insidious in onset and
progressive in nature. It is associated with gradual diminution of vision
for past 1year and gradual decrease in speech for two months .General
examinations showed fasciculations in shoulder and thigh region
associated with wasting of dorsal muscles in hand and foot. Vitals were
normal.CNS examination showed Mini Mental Score of 27/30. The
visual acuity of both eyes showed perception of fingers and light only,
with color blindness. Visual field examination showed field
constriction on both sides. Saccades were slow, horizontal and vertical
gaze were impaired, oculomotor apraxia was present and fundus were
pale with macular dystrophy. Gag reflex were impaired on both sides
with uvula in midline with nasal tone of voice. Other cranial nerve
examinations were normal. Motor system examination showed bulk
was normal, tone increased in all 4 limbs with power 4/5 in all 4 limbs,
left side sustained clonus and right side ill sustained clonus present.
Plantar bilateral flexor. Sensory, extra pyramidal system is normal.
Patient had bilateral axial and appendicular cerebellar signs in the form
of truncal ataxia, finger nose in coordination, dysmetria,
dysdiadochokinesia, tandem walking impaired with broad based gait.
Other systems were normal. Routine lab investigations were normal.
MRI brain showed cerebral and cerebellar atrophy. NCS in upper and
lower limbs were normal. VEP showed poorly formed wave pattern
with both axonal and demyelinating changes. EMG was neurogenic
pattern. This patient had features of amyotrophic lateral sclerosis with
pancerebellar degeneration and ocular cranial nerves and optic nerves
degeneration. We are reporting this rare entity as Coimbatore motor
neuron disease, since it has been reported from this part of land.


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