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Title : A 20 year-old male with gait disturbance
Date : November 15. 2010
Contributed by

Seung Ji Kang, MD

Samsung Medical Center, Seoul, Korea

Patient History
Age/Sex A 20 year-old male
Chief complaint Gait disturbance
Present illness

A 20 year-old man was referred to Samsung Medical Center because of progressive gait disturbance which had been presented for prior 4 months. 4 months before presentation, he had complained the voiding difficulty and visited urologist of local clinic. The doctor inserted foley catheter because of large amount of his residual urine. After several days he visited the neurologist of another hospital. The neurologist did the lumbar puncture and diagnosed him as the tuberculosis meningitis. He was prescribed the anti-tuberculosis medication. However his symptoms such as ataxic gait and urinary difficulty progressed gradually and generalized tonic clonic seizure has been developed. Brain MRI and CSF was obtained repeatedly, after being transferred to SMC

Past medical history

The patient had the hypogammaglobulinemia diagnosed 13 years ago. He had been suffered from recurrent bacterial meningitis and pneumonia since 7 years old. Since 7 years old, he has been received intravenous gamma globulin once a month.

Physical examination

He showed alert mentality and clear orientation. However he had several signs of neurologic deficit such as right 4th cranial nerve palsy, right beating 1st degree nystagmus, ataxic gait and voiding difficulty.

Initial laboratory findings

CBC 6130-11.1-127K,

Chemistry – Na 122mmol/L, Glucose 138mg/dL, Total bilirubin 1.6mg/dL

CRP 2.54 mg/dL

CSF profile

WBC 120/uL (Polymorphonuclear neutrophil 1%, lymphocyte 70%, others 29%)  

Protein 761.2 mg/dl, glucose 124 mg/dl, ADA 15.7 IU/L

Radiologic findings

 
Question - ID Case of the Week ( November 15. 2010 )
What do you think the most likely diagnosis is ?
 
Correct Answer

Chronic Enteroviruses Meningoencephalitis

 
Review

Chronic Enteroviruses Meningoencephalitis in Agammaglobulinemic patients

The enteroviruses have been responsible for persistent, severe, sometimes fatal infections of the CNS in patients with hereditary or acquired defect in B-lymphocyte function. Most reported patients are children with X-linked agammaglobulinemia, although an increasing number of hematopoietic cell transplant patients and patient treated with anti-B-lymphocyte immunotherapy are now being recoreded.

Nervous system manifestations may be totally absent; or mild nuchal rigidity, headache, lethargy, papilledema, seizure disorders, motor weakness, tremors, and ataxia may be present. These neurologic abnormalities may fluctuate in severity, disappear, or steadily progress. The CSF exhibits lymphocytic pleocytosis and a higher protein concentration than is usually seen in cases of acute enterovirus aseptic meningitis. An enterovirus can be repeatedly recovered from the CSF over a period of months to years, usually in high titer. Persistent skeletal muscle involvement causes a dermatomyositis-like syndrome in more than half of these patients, and some also have chronic hepatitis.

Enteroviruses have been recovered from many other sites in these patients, including the brain, lung, liver, spleen, kidney, myocardium, pericardial fluid, skeletal muscle, and bone marrow.  In many patients, possibly most, the disease ends fatally.

Prophylactic use of IVIG serum globulin reduces the risk for acquiring chronic enterovirus infection by these patients. However, IVIG has been less effective in the treatment of chronic enterovirus meningitis, even when using IVIG lots with relatively high concentrations of specific antibody. Some patients have experienced clinical improvement when IVIG has been injected directly into the ventricles, but relapse of infection may occur even after long-term intraventricular IVIG therapy. The experimental antiviral drug pleconaril has been used in this setting, but the reported experience with it is uncontrolled and limited to a small number of patients.

 


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