Transcript Morning Report 8.23.16 – Tolosa Hunt

Noon Report
8/23/16
Pooya Banankhah
HPI
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33 F with no PMH presented with HA, R eye pain, and double vision x 4 days.
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4 days prior to admission: R sided severe headache
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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.
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Also notes periorbital swelling, nausea and chills
Social hx:
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No alcohol, tobacco, drugs.
Currently not working. Lives at home with husband.
Family hx:
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Non-contributory
Physical Exam
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Presenting Vitals:
T: 36.7 °C (Oral) HR: 65 (Monitored) RR: 17 BP: 116 / 67 SpO2: 98%
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Physical Exam:
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Gen: Anxious but in NAD
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HEENT: PERRLA, EOM notable for inability to abduct R eye past midline. Pain w/ lateral and
upward gaze noted in R eye.
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Heart: rrr, no murmurs, rubs or gallops
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Lungs: ctab, no w/r/c
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Ext: warm, well perfused, w/o edema bilaterally
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Skin: No lesions or rashes noted.
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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:
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CBC: WBC:11.6/ Hgb:14.2/ Hct:42.9/ Plt:350
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BMP: Na 136/ K 4.1/ Cl 102/ CO2 102/ BUN 7/ Cr 0.56/ Glu 104
More Labs
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ESR 20, CRP 2.8
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LDH 110
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HgA1c 5.8%
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TSH 3.24
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UA: small blood, trace leuks, negative nitrites, 4WBC, 2RBC
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HIV, RPR, Hep A/B/C panel negative
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SPEP: Normal
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Serum ACE: normal
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SM ab, RNP Ab, Myeloper Ab negative
Imaging
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CT Head:
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No acute intracranial abnormality.
MRI Brain:
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Some very subtle soft tissue thickening and enhancement along the R lateral
cavernous sinus margin extending through the region of R orbital apex
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No evidence of demyelinating disease
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No acute hemorrhage, mass, fluid collection, infarction
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No abnormal parenchymal or leptomeningeal enhancement
Imaging
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MRI Orbit:
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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.
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No dural tail observed to suggest meningioma
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L cavernous sinus unremarkable
Differential Diagnosis
Differential diagnosis:
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Tolosa Hunt Syndrome
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Sarcoidosis
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Lymphoma with meningioma
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Periorbital cellulitis
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TB
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Ophthalmologic migraine
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Poorly controlled DM
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MS
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Myositis
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Duanes syndrome (congenital non-
progressive strabismus)
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Orbital apex syndrome (CN deficit
due to mass lesion near apex)
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Carotid-cavernous fistula or
thrombosis
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ICA dissection
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SCC
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Abscess
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Mucormycosis, actinomycosis
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GCA
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Wegner’s
Lumbar puncture:
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RBC 192, WBC 0
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Cytology: Rare mature lymphocytes and monocytes. Negative for malignant cells
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Flow cytometry: Insufficient sample
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CSF Cx and fungal cx negative
Work up
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No biopsy done by ophtho:
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Meningioma can be diagnosed on imaging and lymphoma can be identified on
cytology from LP
CT Abdomen and thorax:
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No evidence of malignancy or lymphadenopathy
Diagnosis
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Tolosa Hunt Syndrome:
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Treated with Solumedrol 1000mg daily for 3 days followed by Medrol dose pack
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Plan to repeat MRI in 4 weeks
Post-Discharge Follow up
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Follow up in ophtho clinic:
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On methylprednisone 20mg PO daily
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No improvement noted per patient
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No biopsy
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Has neuro follow up
Tolosa Hunt Syndrome
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Definition:
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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:
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One case per million per year
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Same prevalence in men and women
Presentation:
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Pain behind the eye followed by painful ophthalmoplegia
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CN III,IV, VI palsy leading to diplopia
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Unilateral 95% of time
Natural history:
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Benign condition but permanent neurological deficits can occur, relapses occur in at
least 50% of patients and often requiring immunosuppressive therapy
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May resolve spontaneously if left untreated
Tolosa Hunt Syndrome
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Pathogenesis:
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Inflammatory process of unknown etiology
Histopathology:
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Nonspecific inflammation of the septa and wall of the cavernous sinus
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Lymphocyte and plasma cell infiltration
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Giant cell granulomas
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Proliferation of fibroblasts
CN III, IV, VI and superior division of V palsy due to pressure from
inflammation
Tolosa Hunt Syndrome
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Diagnostic Criteria:
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95-100% sensitive, 50% specific
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Unilateral HA
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Granulomatous inflammation of cavernous sinus or orbit on MRI or biopsy
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Paresis of CN III, IV, VI
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Evidence of causation:
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HA preceding oculomotor paresis
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HA around ipsilateral eye
No alternative diagnosis
Imaging Findings
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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
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Treatment:
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Glucocorticoids
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Rapid resolution of pain in 24-72 hours (40%) and within 1 week (78%)
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Improvement in MRI findings in 2-8 weeks
Caveat:
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Lymphoma and vasculitis will also likely respond to steroids