Stiff man syndrome

Two young men, aged 34 and 30 years, developed transient recurrent multifocal neurological symptoms with associated severe headache over a 2-week period. Both had a lymphocytic pleocytosis in their cerebrospinal fluid. Cranial imaging studies were normal. All symptoms resolved without recurrence. Although the cause and pathogenesis are undefined, this selflimited benign neurological syndrome may be more common than previously recognized and has a predilection for young adult men.

Introduction

In 1981, seven patients with a distinctive clinical syndrome consisting of transient recurrent multifocal neurological symptoms, headache, and cerebrospinal fluid (CSF) lymphocytic pleocytosis were described.1 Subsequently, this apparently benign and self-limited syndrome has been reported many times.2 In a recent large series of 50 patients with this syndrome, the age range of affected patients was 14 to 39 years, and 68% of the patients were men .3 Two additional young adult men with this syndrome are reported.

Case Reports

Case 1

A 30-year-old previously healthy man presented with numbness and weakness of the left lower extremity. These symptoms spread to involve the rest of his body and then resolved in 30 minutes. He then developed a severe bifrontal headache with nausea and vomiting. The headache resolved in 6 to 8 hours. His neurological examination was entirely normal by the time he was admitted to the hospital. Head computed tomography without contrast was normal. Complete blood count (CBC) and erythrocyte sedimentation rate were normal. Lumbar puncture was performed and demonstrated a normal opening pressure. CSF white blood cell (WBC) count was 129/(mu)L, of which 94% were lymphocytes. CSF red blood cell (RBC) count was 2/(mu)L. CSF protein was 110 mg/dl. CSF glucose was 59 mg/dl. The patient was discharged from the hospital with a presumptive diagnosis of viral meningitis.

One week later, he again developed left-sided numbness with an associated left visual field deficit, confusion, difficulty speaking, and severe bifrontal headache. His neurological symptoms resolved after 30 minutes. His headache resolved after 6 to 7 hours. His neurological examination was again normal by the time he was admitted to the hospital. Routine blood studies were again normal. Repeat lumbar puncture revealed CSF WBC count of 99/(mu)L, of which 94% were lymphocytes, and CSF RBC count of 185/(mu)L. CSF protein was 108 mg/dl. CSF glucose was 54 mg/dI. Viral cultures were negative. CSF cryptococcal antigen was negative. Serum VDRL was nonreactive. Serum Lyme disease and human immunodeficiency virus antibodies were negative. Cranial magnetic resonance imaging and angiography were normal. Electroencephalography was normal. The patient received no treatment and had no further neurological symptoms.

Case 2

A 34-year-old man with a history of "tension" headaches presented with a 1-week history of three separate episodes of neurological symptoms persisting for about 1 hour followed by a severe headache lasting 7 to 8 hours. These transient neurological symptoms included numbness and tingling of both lower extremities, right-sided weakness, facial asymmetry, and difficulty expressing words. His neurological examination at the time of admission to the hospital was entirely normal. Head computed tomography without contrast was normal. CBC and erythrocyte sedimentation rate were normal. Lumbar puncture was performed and demonstrated a normal opening pressure. CSF WBC count was 240/(mu)L, of which 97% were lymphocytes. CSF RBC count was (mu)L. CSF protein was 206 mg/dl. CSF glucose was 51 mg/dl. CSF cryptococcal antigen was negative. Serum VDRL was nonreactive. Serum Lyme disease antibodies were negative. Cranial magnetic resonance imaging was normal. The patient was discharged from the hospital with a presumptive diagnosis of viral meningitis.

He experienced two more episodes of transient focal neurological symptoms and headache during the next week and then had no further episodes of focal neurological symptoms.

Discussion

The transient syndrome of focal neurological deficits, headache, and CSF lymphocytosis is a clinically dramatic and characteristic entity,2 Focal neurological symptoms in viral meningitis are distinctly unusual, but they are distinctly characteristic of this syndrome .2 Despite extensive searches, to date, no specific virus has been implicated in the pathogenesis of this syndrome.' The report of a series of 50 patients, seen over several years at a few medical centers, suggests that this clinical syndrome may not be rare.' The mean duration of symptoms in this syndrome is about 2 weeks 2 and the maximum duration of symptoms has been 3 months.' Characteristically, the neurological symptoms in these patients vary with each episode.3 The most frequent neurological symptoms are aphasia, focal sensory symptoms, focal weakness, and visual disturbances.2 1 The epidemiological observation that two-thirds of the patients in that large series were young adult men,' as were the two patients in this report, makes this syndrome particularly relevant to military health care providers. The benign prognosis of this syndrome is important to recognize. 1-3

Although similar, this syndrome is quite distinct from Mollaret's meningitis, or benign recurrent aseptic meningitis.2 In Mollaret's meningitis, the recurrent episodes of meningitis occur over a period of years, focal neurological deficits are rare, stiff neck is common, and the CSF leukocyte count may be elevated to several thousand.' In this transient syndrome of focal neurological deficits, headache, and CSF lymphocytosis, the recurrent episodes of meningitis are transient, recurring only over a period of a few months; focal neurological deficits are characteristic, not rare@ stiff neck is uncommon; and the CSF leukocyte count is usually not over a couple of hundred.1-3

References

1. Bartleson JD, Swanson JW, Whisnant JP: A migrainous syndrome with cerebrospinal fluid pleocytosis. Neurology 1981; 31: 1257-62.

2. Berg MJ, Williams LS: The transient syndrome of headache with neurologic deficits and CSF lymphocytosis. Neurology 1995; 45: 1648-54.

3. Gomez-Aranda F, Canadillas F, Marti-Masso JF, et al: Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis: a report of 50 cases. Brain 1997; 120: 1105-13.

Guarantor: CAPT Jack E. Riggs, MC USNR

Contributors: Shyam S. Moudgil, MD*; CAPT Jack E. Riggs, MC USNR*^ ^^

Departments of *Neurology, ^Medicine, and ^^Community Medicine, West Virginia University School of Medicine, Morgantown, WV 26506-9180.

The opinions and assertions contained herein are those of the authors and do not necessarily reflect those of the Navy Medical Department or the Department of Defense.

This manuscript was received for review in January 1999. The revised manuscript was accepted for publication in April 1999.

Reprint & Copyright (C) by Association of Military Surgeons of U.S., 2000.

Copyright Association of Military Surgeons of the United States Feb 2000
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