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:  % 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 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