Abstract

Tolosa-Hunt Syndrome (THS) is a rare disorder characterized by severe unilateral periorbital headache, painful eye movements, and double vision. This syndrome affects males and females equally in their forties to fifties. THS is caused by granulomatous inflammation of the cavernous sinus, usually involving CN III, IV, and VI. We herein report a case of Tolosa-Hunt Syndrome in an 18-year-old female who was initially misdiagnosed as idiopathic intracranial hypertension. Initial treatment was ineffective and the delay may have allowed spread of the disease to involve CN V and VII. This case highlights the pitfalls which can lead to a delay in diagnosis and treatment.

Highlights

  • Tolosa-Hunt syndrome is a rare disorder with an estimated annual case of one individual per million per year [1]

  • Tolosa-Hunt Syndrome (THS) is a rare disorder characterized by severe unilateral periorbital headache, painful eye movements, and double vision

  • We report a case of Tolosa-Hunt Syndrome in an 18-year-old female who was initially misdiagnosed as idiopathic intracranial hypertension

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Summary

Introduction

Tolosa-Hunt syndrome is a rare disorder with an estimated annual case of one individual per million per year [1]. An 18-year-old female with a history of morbid obesity presented for evaluation of an intractable left-sided headache associated with left facial numbness, left eyelid ptosis and diplopia. Three weeks prior to admission, she had a negative noncontrast CT head scan and a negative contrast enhanced MRI brain She was noted to have an increased intracranial pressure with lumbar puncture revealing 35 cm cerebral spinal fluid (CSF) opening pressure. On presentation to our facility, she was suffering from a five-week long unremitting left periorbital stabbing headache She recalled it was unrelieved by her initial spinal tap. A repeat lumbar puncture revealed an opening pressure of 20 cm CSF with 66 lymphocytes consistent with an inflammatory disorder (see Table 1) Her history, physical exam, CSF findings and neuroimaging led to the diagnosis of Tolosa-Hunt syndrome.

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