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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.
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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.
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?
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.