A 20-Year-Old Woman With Headache and Transient Numbness

A 20-year-old woman with a history of migraine presented with 4 days of progressive ptosis, discoordination, paresthesias in her hands and feet progressing to her forearms and shins, and diplopia after an upper respiratory tract illness the prior week. Laboratory evaluation at this time was notable for a positive immunoglobulin (Ig)G Lyme antibody (negative IgM), antinuclear antibody (ANA) 1:80 in a speckled pattern, and a positive GD1b antibody. Lumbar puncture (LP) showed white blood cells 30/mL (87% lymphs), red blood cells 3/mL, protein 68 mg/dL, glucose 49 mg/dL, and a negative Gram stain and bacterial culture. Brain magnetic resonance imaging (MRI) was normal. She was diagnosed with Miller-Fisher variant Guillian-Barre Syndrome and treated with a 5-day course of intravenous Ig, after which her symptoms markedly improved. She was evaluated in the clinic 3 times over the course of the next 4 months, and was found to have a normal neurologic examination except for absent reflexes at each clinic visit, and was otherwise asymptomatic. She was then lost to follow-up. Two-and-a-half years later, she again sought medical attention after a transient sensory disturbance which she described as numbness that began in the roof of her mouth with progression over the course of 10 minutes to involve her entire face and left arm. The numbness lasted 1 hour and then fully resolved. She had experienced a minor headache 2 hours prior that was alleviated by caffeine. Neurologic examination at this time was normal, but a brain MRI showed abnormal leptomeningeal enhancement (Figure 1), and a subsequent LP showed a lymphocytic pleocytosis with a markedly elevated protein and low glucose (Table 1). She underwent an extensive evaluation but no cause for the leptomeningeal enhancement on MRI or the abnormal cerebrospinal fluid (CSF) profile was identified. Two months later, she began to develop progressively worsening vertigo, headache, nausea, and diplopia. The MRI showed new enhancing lesions in the brain and enhancement of the leptomeninges along the entire length of the spinal cord (Figure 1). Evaluation of the spinal fluid at this time again showed CSF pleocytosis and hypoglycorrhacia with an opening pressure of 14 cm water (Table 1), but a cause for these abnormal radiologic and CSF findings could not be identified. Neurosurgical evaluation deemed a biopsy of the affected area would subject the patient to an unacceptably high risk of serious permanent neurologic deficit, and therefore biopsy was deferred. She was placed on empiric treatment for tuberculous meningitis with rifampin, isoniazid, pyridoxine, ethambutol, and a steroid taper. Her symptoms improved rapidly and she was asymptomatic over the course of the next 2 months, with an interval LP demonstrating improvement in the CSF profile (Table 1), and brain MRI showing near resolution of her leptomeningeal enhancement. Unfortunately, after completing her steroid taper she developed nausea, vomiting, and a headache that was worse when lying in the recumbent position. The MRI showed worsening hydrocephalus with return of contrast enhancement of the basilar leptomeninges, as well as the presence of fluid in the maxillary sinus with mucosal thickening. A right inferior nasal turbinate biopsy was unrevealing. Lumbar puncture again showed a CSF lymphocytic pleocytosis with elevated protein and low glucose (Table 1). She was restarted on dexamethasone. One month later, she had a generalized tonic-clonic seizure that was witnessed by her boyfriend. The MRI showed worsening hydrocephalus, and a LP revealed an elevated opening pressure of 40 cm water. A ventriculoperitoneal (VP) shunt was placed at this time to