Horner's syndrome and InterNuclear Ophthalmoplegia

2 Clinical ocular signs: 

2 Clinical ocular signs Monday unit Dr. Ankit Raiyani

1. Horner's syndrome in a case of CV junction anomaly: 

1. Horner's syndrome in a case of CV junction 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: 

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

Slide 4: 

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

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Basilar invagination and sup migration of odontoid process causing compression of cervicomedullary junction, occipitalization of atlas vertebra

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Syringohydromyelia extending from C2- D9

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Diagnosis- CV junction anomaly (Basilar Invagination Group A) with dorsal syringohydromyelia(C2-D9)

Horner’s syndrome: 

Horner’s syndrome Ptosis Miosis Anhidrosis Loss of ciliospinal reflex Heterochromia iridis (if congenital)

Sympathetic system: 

Sympathetic system 1 st order neurons- from hypothalamus to spinal cord 2 nd order neurons- from intermediolateral grey column to paravertebral sympathetic ganglion chain 3 rd order neuron – from paravertebral sympathetic ganglia to different organs Although pathway starts in hypothalamus, there is considerable ipsilateral cortical control

Localization of Horner's syndrome : 

Localization of Horner's syndrome Site of lesion Etiology Extent of Anhidrosis Cerebral Hemispheric lesions Massive infarction Hemispherectomy Thalamic infarcts Ipsilateral face, upper forequarter, upper limb Brainstem lesions (may be a/w pain temp loss over contralateral side) Brain stem infarcts Multiple sclerosis Pontine gliomas Brainstem encephalitis Ipsilateral face, upper forequarter, upper limb Cervical cord lesions ( contralateral sensory loss, ipsilat LMN weakness) Syringomyelia Gliomas, ependymomas Central cord syndrome(post spinal cord trauma) Ipsilateral face, upper limb Radiculopathy ( C8, T1) Malignancy( 1 o & 2 o ) Cervical rib Klumpke’s paralysis Pancoast tumor Ipsilateral face Complete Partial- only upper part Sympathetic chain Malignancy Iatrogenic Rare

Case: 

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

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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 VII th 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

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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

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Pseudo Internuclear Ophthalmoplegia

Internuclear ophthalmoplegia: 

Internuclear ophthalmoplegia Disorder of conjugate lateral gaze two types- Anterior INO Posterior INO

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a) Anterior INO 1 Eyes are divergent Paralysis of both medial recti Seen in midbrain infarcts, multiple sclerosis 1 Crane TB, et al. Analysis of characteristic eye movement abnormalities in internuclear ophthalmoplegia. Arch Ophthalmol 1983; 101:206-210

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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

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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 even after closure of C/L eye

Thank you: 

Thank you