A 25-year-old right-handed woman was admitted to our hospital with a 3-week history of intermittent non-throbbing bilateral headache involving frontal and temporal lobes, accompanied by nausea, vomiting, left upper extremity numbness and weakness, left-sided tongue and perioral numbness, and involuntary movements of the left upper extremity. There were five episodes with complete recovery in the last 3 weeks, of which two episodes were without any neurologic deficit and three episodes were preceded by transient symptoms. The numbness and weakness lasted for about 10 to 20 mins and were ensued by headache and involuntary movements of the left upper extremity. The headache lasted approximately 2-9 hrs and was graded as 8/10 in the VAS (with 0 indicating no headache and 10 the most severe headache). The involuntary movements of the left upper extremity occurred in two episodes and lasted for about 1 to 2 hrs. The involuntary movements of the left upper extremity and headache occurred simultaneously after numbness and weakness of the left upper extremity, and the duration of involuntary movement was shorter than that of the headache. She was conscious during the episodes. The involuntary movements were chorea of the left upper extremity, which presented as irregular, arrhythmic and brief movements of the left hand and forearm. The chorea of the left hand was more obvious than the forearm, and the movement was remarkable during the voluntary movements. Cough, yellow sputum and snot were also reported, but no fever. She had no history or relevant family history of migraine. She had a regular menstrual cycle and no history of previous pregnancy. She did not take oral contraceptives or any other medication before the headache. She was Han Chinese and worked as an office clerk.
Upon admission, she had no symptom and the neurological examinations were unremarkable. Fundus examination revealed no papilledema or any other abnormalities. The routine blood tests and autoimmune tests were both normal. Also, the cerebral magnetic resonance imaging (with contrast medium) and angiography did not show any abnormality (Figure 1).
The first lumbar puncture at an external hospital showed intrathecal cerebrospinal fluid (CSF) opening pressure of 20cmH2O and prominent lymphocytic (90%) pleocytosis with the cell count of 114/microL.
The second lumbar puncture conducted in our hospital revealed slightly increased CSF opening pressure of 30cmH2O, and confirmed the pleocytosis with cell count of 54/microL (lymphocytes 81%). There was also increased level of protein with 5.04 g/L, but normal glucose level. Serological tests were positive for lgG coxsackie virus and lgG varicella-zoster, and CSF tests of IgM/G virus were negative. Mycobacterium tuberculosis and parasites were not detected in CSF, and oligoclonal bands were not detected as well. Electroencephalogram of asymptomatic period showed no abnormality.
Initial treatment with non-steroidal anti-inflammatory drugs, ganciclovir, mannitol and anti-epileptic medications at an external hospital did not halt the recurrence of the symptoms. During admission at our hospital, she was given ganciclovir initially, but she developed two new episodes of headache (VAS 7/10) with focal neurological symptoms, including numbness, weakness and involuntary movements of the left upper extremity. Chorea of the left hand lasted for 30-45 mins and headache for about 2 hrs. The left upper extremity weakness was graded as 4/5 using the Medical Research Council grading system. The characteristics of the episodes were similar to that described above. Meningeal irritation sign was absent during the attack.
The third lumbar puncture showed 30cmH2O of CSF opening pressure and lymphocytosis (74%). She was treated with intravenous methylprednisolone with initial dose of 120 mg/day which was tapered to 40 mg/day within 7 days. The fourth CSF examination after the administration of methylprednisolone for 7 days showed CSF opening pressure of 23cmH2O and cell number of 24/microL with 74% of lymphocytes. She did not experience any recurrence of the symptoms, and the oral methylprednisolone was discontinued gradually within 1 month. After about 1 month, she was discharged from our hospital. The final and fifth CSF examination showed CSF opening pressure of 17cmH2O and cell count of 12/microL with 79% of lymphocytes. The five CSF examinations are summarized in Table 1. At 1-year follow-up, she denied any recurrence of the symptoms. Written informed consent from the patient was obtained to perform invasive procedures and publish data. The ethics committee of our institute approved the publication of the case details.