Transcript of "Horner's syndrome and Internuclear ophthalmoplegia"
Two Clinical ocular signs Monday unit Dr. Ankit Raiyani
1. Horners syndrome in a case of CVjunction anomaly• 26 Year Male/Hindu/Right handed/ R/o Bandra/Street hawker, head load worker• Presented with complaints of – Progressive weakness in right upper limb since 5 years (starting with difficulty in holding small objects in right hand….gradually progressing to involve whole right UL over next 2 years….now patient cannot move his right UL since last 3 years) – Tingling sensations in right upper limb since 5 years (continuous, not affected by movements of neck, coughing) – Difficulty in walking due to tightness in right lower limb since 3 months (difficulty in bending his right knee while walking)• On enquiry patient also gave H/O – Twitching in right shoulder and right arm – Absence of sweating over right side of face, right forequarters, and right upper limb.
Relevant clinical findings• Exaggerated Cervical lordosis, scoliosis with convexity to right• Anhidrosis involving right side of face, right upper forequarters, right UL• Right pupil 2mm RTL slow to relax in dark• Left pupil 3 mm RTL.• Tongue fasciculations with atrophy of right side of tongue• LMN type weakness(wasting, flaccidity, areflexia) in right upper limb• Spasticity and hyperreflexia in right lower limb• Loss of touch, pain, temp in right shoulder and right UL in c5- c8 dermatomal areas with preservation of post column sensations
• Clinical Diagnosis- 26/M with right UL LMN paralysis with right LL UMN involvement with ipsilateral sensory loss (involving touch, pain, temp) from C3-C8 dermatome with ipsilateral Horner’s syndrome
Basilar invagination and sup migration ofodontoid process causing compression ofcervicomedullary junction, occipitalization ofatlas vertebra
• Diagnosis- CV junction anomaly (Basilar Invagination Group A) with dorsal syringohydromyelia(C2-D9)
Horner’s syndrome• Ptosis• Miosis• Anhidrosis• Loss of ciliospinal reflex• Heterochromia iridis (if congenital)
Sympathetic system• 1st order neurons- from hypothalamus to spinal cord• 2nd order neurons- from intermediolateral grey column to paravertebral sympathetic ganglion chain• 3rd order neuron – from paravertebral sympathetic ganglia to different organsAlthough pathway starts in hypothalamus, there is considerable ipsilateral cortical control
Case• 20yr/male/college student/unmarried• Referred with difficulty in standing and walking due to imbalance, tendency to fall on either side 2 days• Clinical Exam on presentation- Sensory ataxia, areflexia. Power was 4+ diffusely at first examination.• Diagnosed as Miller Fisher Syndrome initially• Past history- acute pharyngitis 1 week before onset of paralysis
• Patient progressively developed quadriparesis, dysphagia to solids and liquids with nasal regurgitation of liquids, diplopia, vertigo, tendency to fall…. Over 4 days• CNS exam AT 5 DAYS- • CN- EOM- B/L INO+, B/L VIIth nerve LMN weakness, B/L IX/X nerve involvement( decreased soft palate movements, absent gag weak cough) • Hypotonia, areflexia involving all 4 limbs • Power – 3/5 in UL, 3+/5 in LL, • No sensory loss • Cerebellar- B/L past pointing + • No involuntary movements
• EMG/NCV- early demyelinating polyradiculoneuropathy s/o Guillain Barre Syndrome• Diagnosis- Miller-Fisher variant of GBS• Treatment- – Plasmapheresis - total 7 cycles – IV Immunoglobulin 2Gm/kg body weight over 5 days
Internuclearophthalmoplegia• Disorder of conjugate lateral gaze• two types- – Anterior INO – Posterior INO
a) Anterior INO1 • Eyes are divergent • Paralysis of both medial recti • Seen in midbrain infarcts, multiple sclerosis1 Crane TB, et al. Analysis of characteristic eye movement abnormalities in internuclearophthalmoplegia. Arch Ophthalmol 1983; 101:206-210
b). Posterior INO• There is a painless onset of visual disturbance,• No diplopia in primary gaze• Medial longitudinal Fasciculus involved• the affected eye shows impairment of adduction when both eyes are tested simultaneously• The contralateral eye abducts, however with nystagmus.• This is reversed when patient looks to opposite side in bilateral involvement
Pseudo INO• Involvement of cranial nerves or neuro- muscular junction• MLF is not involved• Seen in Myasthenia gravis, GBS• Differentiating point from true INO- absence of adduction of affected eye evenafter closure of C/L eye