Motivation: This year, I have seen quite a number of middle aged otherwise healthy patients present with shingles. Usually, initial questioning leads to some complaints of headaches, which results in a lumbar puncture showing a few cells. Now the possibilities open. Could the patient have VZV vasculitis? I was once asked this question, and I had no clue what VZV vasculitis looked like.
Paper: Nagel, M.A., Cohrs, R.J., Mahalingam, R., et. al. "The varicella zoster virus vasculopathies." Neurology (2008); 70: 853-860.
Methods: Review of thirty patients (previous published cases and unpublished cases) with serologic or PCR proof of positive VZV and neurologic signs or symptoms attributed to VZV infection by the reviewing authors.
Results:
Cohort: Of the thirty patients, the age ranged from one year to 88 years with fifty percent of patients male and the rest female. Of the thirty patients, eleven patients had important comorbid disorders - 2 with AIDS, 3 with HIV, 2 with leukemia, 1 with CREST syndrome, 1 with lymphoma, 1 with decreased CD4 count, and 1 on immunosuppression for lupus and rheumatoid arthritis.
Clinical Features: In total, of 30 patients, nineteen (63%) had rash, twenty (67%) had CSF pleocytosis of >5 wbc. Average interval between onset of rash and neurological symptoms was 4.1 months. Of the eleven immunocompromised patients, six (54%) had rash, nine (82%) had CSF pleocytosis, six (54%) had positive VZV PCR, and eleven (100%) had anti-VZV IgG antibody. In the 19 immunocompentent, thirteen (68%) had rash, eleven (58%) had CSF pleocytosis, three (16%) had positive PCR, and seventeen (89%) had positive anti-VZV IgG in CSF. PCR was positive more frequently in the immunocompromised. Of note, all patients with CSF anti-VZV IgG had reduced serum to CSF ratio.
Imaging: Of the entire cohort, 29 (97%) had abnormal brain imaging on MRI or CT scans. Descriptively, these lesions often were centered in white matter and gray-white junction. Of 23 who had vascular imaging, sixteen (70%) showed abnormalities. Larger artery disease occurred exclusively in four (13%) while mixed small and large vessel and small vessel involvement occurred in fifteen (50%) and eleven (37%).
Discussion: This case-series of VZV vasculopathy suggests that it defies some of our conventional thoughts about vasculitis. About 30% had no pleocytosis, and 37% had no preceding rash to suggest shingles. Also, despite our initial impression that severe disease is more common in the immunocompromised, nineteen patients had no known immune suppressing conditions. Finally, VZV PCR has little diagnostic sensitivity, and serology remains the better diagnostic test. I think that overall, what this series suggests is that for multi-focal strokes of uncertain etiology, VZV should always be on the differential.
Paper: Nagel, M.A., Cohrs, R.J., Mahalingam, R., et. al. "The varicella zoster virus vasculopathies." Neurology (2008); 70: 853-860.
Methods: Review of thirty patients (previous published cases and unpublished cases) with serologic or PCR proof of positive VZV and neurologic signs or symptoms attributed to VZV infection by the reviewing authors.
Results:
Cohort: Of the thirty patients, the age ranged from one year to 88 years with fifty percent of patients male and the rest female. Of the thirty patients, eleven patients had important comorbid disorders - 2 with AIDS, 3 with HIV, 2 with leukemia, 1 with CREST syndrome, 1 with lymphoma, 1 with decreased CD4 count, and 1 on immunosuppression for lupus and rheumatoid arthritis.
Clinical Features: In total, of 30 patients, nineteen (63%) had rash, twenty (67%) had CSF pleocytosis of >5 wbc. Average interval between onset of rash and neurological symptoms was 4.1 months. Of the eleven immunocompromised patients, six (54%) had rash, nine (82%) had CSF pleocytosis, six (54%) had positive VZV PCR, and eleven (100%) had anti-VZV IgG antibody. In the 19 immunocompentent, thirteen (68%) had rash, eleven (58%) had CSF pleocytosis, three (16%) had positive PCR, and seventeen (89%) had positive anti-VZV IgG in CSF. PCR was positive more frequently in the immunocompromised. Of note, all patients with CSF anti-VZV IgG had reduced serum to CSF ratio.
Imaging: Of the entire cohort, 29 (97%) had abnormal brain imaging on MRI or CT scans. Descriptively, these lesions often were centered in white matter and gray-white junction. Of 23 who had vascular imaging, sixteen (70%) showed abnormalities. Larger artery disease occurred exclusively in four (13%) while mixed small and large vessel and small vessel involvement occurred in fifteen (50%) and eleven (37%).
Discussion: This case-series of VZV vasculopathy suggests that it defies some of our conventional thoughts about vasculitis. About 30% had no pleocytosis, and 37% had no preceding rash to suggest shingles. Also, despite our initial impression that severe disease is more common in the immunocompromised, nineteen patients had no known immune suppressing conditions. Finally, VZV PCR has little diagnostic sensitivity, and serology remains the better diagnostic test. I think that overall, what this series suggests is that for multi-focal strokes of uncertain etiology, VZV should always be on the differential.
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