two different cases with Horner's syndrome and Pseudo INO

1. Two Clinical ocular signs
Monday unit
Dr. Ankit Raiyani

2. 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.

3. 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

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

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

7. Syringohydromyelia extending from C2- D9

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

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

10. 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 organs
Although pathway starts in hypothalamus,
there is considerable ipsilateral cortical control

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

13. 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

14. • 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

15. • 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

17. Internuclear
ophthalmoplegia
• Disorder of
conjugate lateral
gaze
• two types-
– Anterior INO
– Posterior INO

18. a) Anterior INO1
• 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

19. 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

20. 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

21. Thank you