A 36-year-old UAE national man started having headache about 3 weeks before his admission to our hospital 15 May 2000. He had been to the ER of our hospital twice and to 3 other hospitals during the previous two weeks. He had had, by report, a normal CT. On two occasions during his youth, he had "woken up" in strange situations such as once in the doghouse in the yard of his home. This episode was accompanied by headache. He and his sisters had a history of episodic headaches relieved by over the counter medications of which he had been taking a lot the previous two weeks. On his third visit to our ER a neurological consult was called. His temperature was 38.5 ear. His neurological examination was essentially normal except for early papilledema . There were no signs of meningeal irritation. Lumbar puncture was performed after a normal CT: 400 cells, 95% lymphs, 110 RBC, Protein of 1.9 (190), Glucose 4.5, CK 5, CRP 0. Opening Pressure 190, CP 110. Headache was immediately relieved. He had one dose of acyclovir that evening but this was stopped the next day after a normal MRI and EEG. The following morning his headache was worse and the LP was repeated with same results--headache markedly relieved. His next few days were un-eventful except for spiking fevers which seemed to be going generally downward. He persuaded the physician to allow him home on the morning of the 19 May since no specific therapy was being given in hospital. He had no papilledema at the time of discharge.
Instead of going home he went to the barber's, then to his mother's house where he promptly had a grand mal seizure and was taken to another hospital. He signed out AMA from that hospital and appeared at our hospital in the early morning hours of 20 May. His exam was normal. There was no papilledema but he did have a headache and fever. His new spinal fluid is noted in the CSF chart. He was normal mentally at 2 am. At 6 am he became very confused, unable to follow commands, agitated, trying to get out of bed and requiring the presence of another person to keep him calm. His speech was garbled and there was a suggestion of a right hemiparesis. Acyclovir was restarted. Another LP was performed that day, showing pleocytosis and increased protein like the first LP. An EEG showed bifrontal high voltage delta activity with a background of 6 Hz with left hemisphere slightly slower than the right. An MRI scan was done under general anesthesia and was reported as normal. He was started on Rocephin, Ampicillin, Anti Tbc medications (triple therapy) as well as acyclovir. He required heavy sedation and heavy doses of demerol to keep him barely manageable over the next two days while he continued to spike fevers to 39 C. LABORATORY WORK: NEGATIVES: PCR FOR TBc & HSV on CSF; leptospiral titers; cryptococcal; Echinococcal; HTLV, HIV, ACE 17 (18-55); Bence-Jones protein; ANA; Immunoelectrophoresis; thyroid studies, B12; folic acid. Numerous blood, urine and CSF cultures negative. Urine C&S, urinalysis also negative. Chest x-ray negative. MRI dorsal and LS spine normal. CSF Gram Stain and AFB stain negative on several occasions.
On 24 of May he was more agitated than usual. His pupils were larger and less reactive. General anesthesia was required for an MRI scan which showed developing hydrocephalus. That evening a ventricular shunt to external drainage was inserted with an opening pressure of 270 mm of HOH. It took him about 12 hours to wake up from his surgery but when he did awaken, he was essentially back to his "normal" self from then until discharge. At the time of shunt insertion, decadron was added starting with a bolus of 40 mg and 8 mg Q 6 hours. Also at the time of shunt insertion he was unable to concentrate his urine with a low serum sodium, low urine sodium but normal osms in blood and rising creatinine. Acyclovir was stopped, fluids restricted mildly and he corrected his electrolytes within 36 hours.
From 24 May to 8 June, the ventricular external drainage remained and fluid was examined often (see CSF chart). The shunt exit pressure was gradually increased to 200 mm HOH until 8 June when he was tolerating 200 mm HOH pressure without headache or papilledema or neurological deficit. Decadron was tapered and stopped 8 days prior to removal of his shunt. Anti TBc meds stopped 4 days prior and antibiotics the day after the shunt was removed. He was discharged with a normal neurological exam (but NOT normal CSF!). On 12 June 2000 and seen on two occasions in the office before he went to Germany for a second opinion. When he left for Germany, I still did not know what was his diagnosis. What do you'all think this was?
|SPINAL FLUID RESULTS|
|L = lumbar||V = ventricle|
|4||V||244||69||31||211||0.59||3||<1||0||and Plasma Cells|
I'm worried that Farouk is getting too anxious. Chris Hawkes also thought of idiopathic meningitis (Mollaret's), as Della did when the patient left for Germany. His chest XRay and Angiotensin Converting Enzyme level were normal so sarcoid never became a major concern. Michael Okun's Hashimoto's encephalitis didn't occur to us because we don't know about it. Then there are the cyst troops: Farouk, Suri Rao, Pepe Maestre and Len Cohen, mainly inspired by that cystic appearance in the ventricle. When I first saw it I wanted to go in and extract it, but cooler heads prevailed. I am still waiting for someone on the list to write in and say they have seen such a 'flow phenomenon' before. Ahoy, Bob Fink. Len Cohen and others asked about lesions elsewhere such as liver and muscles. Imaging was done and there was nothing else.
Della has already written: after returning from Germany he became sick with headache, disorientation, slurred speech, and the CSF was still quite abnormal. The day he was readmitted to the hospital here was exactly six weeks from his first admission, and on that day the hospital reported a positive TB culture from his first LP. Two days later Bioscientia in Germany reported positive TB culture on the same specimen from six weeks prior (our lab had sent out a backup culture to Bioscientia).
You've heard that he was started on antituberculous triple therapy at the beginning, with Rocephin and steroids, and he did improve. When the CSF PCR test was negative at Bioscientia we decided to stop the anti-TB therapy, and by this time, with ventricular drainage in place, he didn't look sick and was afebrile (on steroids). A repeat MRI on this readmission still showed the 'flow defect' but now also showed marked contrast enhancement at the base around the right middle cerebral artery. After this admission he developed a left facial weakness, slurred speech, and mild left weakness. Later, density alterations in the right lenticulostriate distribution appeared on MRI.
Now of course he's back on (quadruple) antitubeculous therapy and high dose steroids, and is improving. The last tap three days ago had 100 WBC, 98% lymphs, protein 74mg%, glucose 2.5mmol/L, opening pressure 170mm HOH. Dr. Banu called it TB because of the hydrocephalus, and Dr. Pepe wondered if it was false negative PCR. The doctors in Munich felt if was likely TB, but didn't want to treat until proof was in hand. To me it was a good lesson that TB meningitis can sometimes be relatively mild. I've been used to seeing only more fulminant cases. And a lesson that TB meningitis can occur without abnormal chest XRay, without coexisting immunosuppressive disease or alcoholism. This man is normal mainstream Dubai citizen. And a reminder that the PCR test shouldn't be relied on too heavily. Fortunately the patient is doing well now and hopefully will be on oral medication at home in about a week. We are grateful to patients like this who educate their doctors!
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Revised July 29, 2000
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