Short Case Presentation Dr. Sania Khalid

Background Young female developed quadriplegia over a year Bed-ridden for 2 months Loss of bowel and bladder control We will be showing you few clinical tests performed on this lady.

Name the sign and interpret.

A sagittal T1 weighted MR image scan

MRI cervical and thoracic spine.

Axial T2 weighted MR image showing C4-5 level

MRI cervical and thoracic spine. MRI of cervical spine and thoracic spine showed a bilobed mass like lesion in the left intervertebral foramina at C4-C5 level with an intraspinal component showing severe compressionover the spinal cord at this level. Post Gandolinium sacn showed intense heterogenous enhancement. Impression: was of a dumb-bell shaped neurofibroma at left intervertebral foramina of C4-C5 causing severe external compression over the spinal cord.

Histopathology: On histopathology : Spindle shaped neoplasm, cells having wavy nuclei with tapering ends.Hyper and hypocellular areas were scene. In few areas verrucay bodies present. No mitosis or atypia noted. Most like a benign peripheral nerve sheath tumor or schwanomma.

Case 2 Myriad of deficits: Diagnosing Topsy-turvey Cases Dr. Muhammad Zaman Khan Resident Department of Medicine Jinnah Hospital Lahore

Closely observe this examination and answer following questions Write down three most important findings. What three steps are important in relevant examination? Write down a list of differential diagnosis? How would you investigate this patient?

Observe…………

Left Complete Blepheroptosis

Left Complete Extra-occular Ophthalmoplegia

Anisocoria

Complete loss of vision in left eye Fundoscopic examination is unremarkable Rest of neurological examination is unremarkable except for absent left corneal reflex and loss of left forehead sensations.

Nerves involved Left – II – III – IV – V1 – VI Right XII

Causes of multiple cranial nerve palsies Cavernous sinus Pathologies Orbital apex syndrome Tumors Inflammation (viral, fungal, bacterial, granulomatous, vasculitis) Diabetes Ophthalmoplegic migraine

Workup Complete blood count Electrolytes Glucose and hemoglobin A1C Renal and liver function tests Angiotensin converting enzyme Antinuclear antibody Anti-dsDNA antibody Anti-Sm antibody Antinuclear cytoplasmic antibody Fluorescent treponemal antibody test Lyme serologies Serum protein electrophoresis Erythrocyte sedimentation rate (ESR) C reactive protein

CSF analysis MRI Brain with Gadolinium

Diagnostic criteria for Tolosa Hunt Syndrome One or more episodes of unilateral orbital pain lasting for weeks (untreated) Third, fourth, and/or sixth cranial nerve palsy and/or granuloma detected by magnetic resonance imaging (MRI) or biopsy Cranial nerve palsy begins within two weeks of onset of orbital pain Symptoms resolve within 72 hours when treated with sufficient corticosteroids Other etiologies are excluded by appropriate investigation

Tessitore E, Tessitore A. Tolosa- Hunt syndrome preceded by facial palsy. Headache 2000; 40:393. Adams AH, Warner AM. Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications. Bull Los Angeles Neurol Soc 1975; 40:49.

Treatment Prednisone 80 to 100 mg daily for three days. If the pain has resolved, taper to 60 mg daily, then 40 mg, then 20 mg, then 10 mg every two weeks.

After 1 week

After Few Months

Thank You