1. The document describes a case of a 26-year-old male presenting with progressive weakness in his right upper limb over 5 years and difficulty walking due to tightness in his right lower limb for 3 months. On examination, he showed signs of Horner's syndrome and LMN weakness in his right upper limb with UMN involvement in his right lower limb. 2. Imaging revealed basilar invagination and syringohydromyelia extending from C2-D9. He was diagnosed with a CV junction anomaly (basilar invagination) with dorsal syringohydromyelia. 3. The document then discusses Horner's syndrome, its localization, and

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