Neurology India, Vol. 58, No. 5, September-October, 2010, pp. 820-821
Letter to Editor
Idiopathic intracranial hypertension with complete oculomotor palsy
Arezoo Rezazadeh, Mohammad Rohani
Department of Neurology, Iran University of Medical Sciences, Tehran, Iran
Date of Acceptance: 22-Jul-2010
Code Number: ni10239
Idiopathic intracranial hypertension (IIH) is a syndrome of increased intracranial pressure without a space occupying lesion.  We report a unique patient with IIH presenting with complete oculomotor palsy.--
An otherwise healthy 21-year-old woman with a past medical history of facial acne treated with isotretinoin presented with two weeks of progressive generalized headache, vomiting, blurred vision and tinnitus. There was no history of fever or past history of headache. On examination she was conscious and alert. Vital signs were normal. Blood pressure was 100/80. She had right ptosis with limitation of all right eye movements except for abduction. Right pupil was dilated and not reacting to light [Figure - 1]a. Left pupil was normal. Visual acuity was one meter finger count OD and 20/80 OS. Fundus examination showed bilateral papilledema without hemorrhages. Motor and sensory systems were normal. There were no signs of meningeal irritation. Routine labs were normal. Lumbar puncture revealed an opening pressure of 400 mm H 2 O with normal composition. Imaging studies including brain computerized tomography (CT) scan, magnetic resonance imaging (MRI), MR-venography, CT angiography were normal [Figure - 2]. The patient was treated with multiple lumbar punctures and acetazolamide and was discharged a few days later on acetazolamide. Follow up exams, one and six months later, showed dramatic improvement of symptoms and signs including headache, papilledema, ophthalmoplegia and dilated pupil [Figure - 1]b and c. Visual acuity improved partially after one year of follow up.
The diagnostic criteria for IIH has been revised and these include:  (1) symptoms if present, reflect only those of increased intracranial pressure or papilledema; (2) signs attributable only to increased intracranial pressure and papilledema; (3) documented elevated intracranial pressure, during lumbar puncture measured in the lateral decubitus position; (4) normal cerebrospinal fluid composition; (5) no ventriculomegaly, mass, structural vascular lesion on neuroimaging studies. (MRI or contrast-CT for typical patients and MRI and MRV for all others); and (6) If no other cause (including medications) of intracranial hypertension is identified, the syndrome is termed Idiopathic intracranial hypertension.
In most cases the cause is unknown but can include certain drug ingestion or systemic inflammation and metabolic disease.  The present patient satisfied all the diagnostic criteria of IIH. Sixth nerve palsy is reported to occur in 10-40% of patient in most series of IIH,  which is a nonlocalizing sign of increased intracranial pressure and can be unilateral or bilateral.  There has been only one case report of fourth nerve palsy being associated with IIH.  Oculomotor palsy is an uncommon association seen in IIH, ,,,, in which all has been attributed to raised intracranial pressure. Pupillary fibers were spared in all the reported cases. However, the present patient is the first one in whom the pupil was affected too. This finding led us to investigate for secondary causes. The results were all normal so we concluded that third nerve palsy, involving pupil fibers, could be a nonlocalizing sign of IIH, although secondary causes exclusion is recommended.
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