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Download Morning Report 8.23.16 – Tolosa Hunt
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Noon Report 8/23/16 Pooya Banankhah HPI 33 F with no PMH presented with HA, R eye pain, and double vision x 4 days. 4 days prior to admission: R sided severe headache 2 days prior to admission: headache developed into sudden-onset R sided eye pain 10/10, radiating to her R jaw, associated with double vision, and worse with eye movement. Also notes periorbital swelling, nausea and chills Social hx: No alcohol, tobacco, drugs. Currently not working. Lives at home with husband. Family hx: Non-contributory Physical Exam Presenting Vitals: T: 36.7 °C (Oral) HR: 65 (Monitored) RR: 17 BP: 116 / 67 SpO2: 98% Physical Exam: Gen: Anxious but in NAD HEENT: PERRLA, EOM notable for inability to abduct R eye past midline. Pain w/ lateral and upward gaze noted in R eye. Heart: rrr, no murmurs, rubs or gallops Lungs: ctab, no w/r/c Ext: warm, well perfused, w/o edema bilaterally Skin: No lesions or rashes noted. Neuro: A&Ox3, CN normal except for diplopia present in right eye but not left, EOMI intact except for inability to abduct right eye past midline, Vision 20/20 in both right and left eye, otherwise normal neuro exam. Labs: CBC: WBC:11.6/ Hgb:14.2/ Hct:42.9/ Plt:350 BMP: Na 136/ K 4.1/ Cl 102/ CO2 102/ BUN 7/ Cr 0.56/ Glu 104 More Labs ESR 20, CRP 2.8 LDH 110 HgA1c 5.8% TSH 3.24 UA: small blood, trace leuks, negative nitrites, 4WBC, 2RBC HIV, RPR, Hep A/B/C panel negative SPEP: Normal Serum ACE: normal SM ab, RNP Ab, Myeloper Ab negative Imaging CT Head: No acute intracranial abnormality. MRI Brain: Some very subtle soft tissue thickening and enhancement along the R lateral cavernous sinus margin extending through the region of R orbital apex No evidence of demyelinating disease No acute hemorrhage, mass, fluid collection, infarction No abnormal parenchymal or leptomeningeal enhancement Imaging MRI Orbit: Enhancing T1 isointense soft tissue extending toward the right orbital apex and to the origin of the right lateral rectus muscle measuring approximately 8.9x6.8x14.8mm. No dural tail observed to suggest meningioma L cavernous sinus unremarkable Differential Diagnosis Differential diagnosis: Tolosa Hunt Syndrome Sarcoidosis Lymphoma with meningioma Periorbital cellulitis TB Ophthalmologic migraine Poorly controlled DM MS Myositis Duanes syndrome (congenital non- progressive strabismus) Orbital apex syndrome (CN deficit due to mass lesion near apex) Carotid-cavernous fistula or thrombosis ICA dissection SCC Abscess Mucormycosis, actinomycosis GCA Wegner’s Lumbar puncture: RBC 192, WBC 0 Cytology: Rare mature lymphocytes and monocytes. Negative for malignant cells Flow cytometry: Insufficient sample CSF Cx and fungal cx negative Work up No biopsy done by ophtho: Meningioma can be diagnosed on imaging and lymphoma can be identified on cytology from LP CT Abdomen and thorax: No evidence of malignancy or lymphadenopathy Diagnosis Tolosa Hunt Syndrome: Treated with Solumedrol 1000mg daily for 3 days followed by Medrol dose pack Plan to repeat MRI in 4 weeks Post-Discharge Follow up Follow up in ophtho clinic: On methylprednisone 20mg PO daily No improvement noted per patient No biopsy Has neuro follow up Tolosa Hunt Syndrome Definition: Episodic orbital pain associated with paralysis of one or more of the CN III, IV, VI due to granulomatous inflammation of the cavernous sinus Epidemiology: One case per million per year Same prevalence in men and women Presentation: Pain behind the eye followed by painful ophthalmoplegia CN III,IV, VI palsy leading to diplopia Unilateral 95% of time Natural history: Benign condition but permanent neurological deficits can occur, relapses occur in at least 50% of patients and often requiring immunosuppressive therapy May resolve spontaneously if left untreated Tolosa Hunt Syndrome Pathogenesis: Inflammatory process of unknown etiology Histopathology: Nonspecific inflammation of the septa and wall of the cavernous sinus Lymphocyte and plasma cell infiltration Giant cell granulomas Proliferation of fibroblasts CN III, IV, VI and superior division of V palsy due to pressure from inflammation Tolosa Hunt Syndrome Diagnostic Criteria: 95-100% sensitive, 50% specific Unilateral HA Granulomatous inflammation of cavernous sinus or orbit on MRI or biopsy Paresis of CN III, IV, VI Evidence of causation: HA preceding oculomotor paresis HA around ipsilateral eye No alternative diagnosis Imaging Findings Axial imaging without (left) and with (right) enhancement demonstrates nonspecific fullness involving the left cavernous sinus, consistent with Tolosa-Hunt syndrome within the context of the history. Tolosa Hunt Syndrome Treatment: Glucocorticoids Rapid resolution of pain in 24-72 hours (40%) and within 1 week (78%) Improvement in MRI findings in 2-8 weeks Caveat: Lymphoma and vasculitis will also likely respond to steroids
