Cerebral Vasculitis and Tolosa Hunt Syndrome

Rheumatology Morning Report

Today's case involved a young woman with known p-ANCA-associated vasculitis with manifestations of pulmonary hemorrhage, end-stage renal disease, and possible CNS involvement.  She presented to hospital with a history of headache and oculomotor nerve palsy (albeit sparing her extraocular movements) as well as trigeminal nerve (V1) involvement.  Her CT Angiogram was consistent with diffuse vasculitis of the CNS, while her MRI was much less impressive.  Lumbar puncture (performed several days following admission, after antibiotics) had mild pleiocytosis (8 lymphocytes) and hypoglycorrhachia (low glucose in spinal fluid).  Cultures have been negative to date despite impressive fevers on admission.  At this point, she is being treated with steroids for a presumed inflammatory CNS condition.

Learning points:

-We discussed vasculitis and the diagnostic elements and associations including cytoplasmic and perinuclear ANCA

-We discussed the systemic fibrosis that can take place when patients with ESRD are given gadolinium repeatedly

-We discussed the differential diagnosis of a third nerve palsy (CNIII) and that, if the pupil is spared, the patient typically has an infarction of the central portion of the nerve rather than a compressive etiology – a common cause of this would be diabetes

-We discussed Tolosa-Hunt syndrome, a granulomatous inflammation of the cavernous sinus which can cause damage to multiple cranial nerves – typically treated with immune suppression and can be an HIV-associated illness

-We discussed drug-induced lupus (usually a benign illness associated with drugs like Hydralazine) but also drug-induced vasculitis which can be quite severe and organ-damaging caused by drugs such as PTU and methimazole

-We discussed the rarity of CNS vasculitis and that a normal cerebrospinal fluid and a normal MRI brain has an extremely high negative predictive value to rule out this challenging-to-diagnose condition

Further Reading:

Jennette, J. C., & Falk, R. J. (1997). Small-vessel vasculitis. New England Journal of Medicine, 337(21), 1512-1523.

Hajj-Ali, R. A., & Calabrese, L. H. (2009). Central nervous system vasculitis. Current opinion in rheumatology, 21(1), 10-18.

Kline, L. B., & Hoyt, W. F. (2001). The Tolosa-Hunt syndrome. Journal of Neurology, Neurosurgery & Psychiatry, 71(5), 577-582.