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Report of a Case and Review of the Literature of Progressive Multifocal Leukoencephalopathy With Other Inherited Immunodeficiency States

Debra A. Katz, MD; Joseph R. Berger, MD; Brian Hamilton, MD, PhD; Eugene O. Major, PhD; M. Judith Donovan Post, MD

Arch Neurol. 1994;51(4):422-426.

Abstract
Objective
To describe the occurrence of progressive multifocal leukoencephalopathy (PML) in association with Wiskott-Aldrich syndrome, an X-linked recessive disorder with impairment of both cellular and humoral immunity.

Design
A detailed analysis of this patient's clinical illness, immunologic factors, neuroradiographic findings, and brain histopathologic condition was undertaken. The medical literature on PML complicating congenital immunodeficient states was also reviewed.

Setting
A 1500-bed, university-affiliated, public health hospital.

Patient
A 15-year-old boy with Wiskott-Aldrich syndrome. His neurologic illness was heralded by dysarthria and right-sided weakness and the diagnosis was established by brain biopsy specimen. Survival from the time of onset of PML was 10 months.

Conclusion
Although PML typically occurs in the setting of severe acquired cellular immunodeficiency, often as a consequence of acquired immunodeficiency syndrome, organ transplantation, and leukemia and lymphoma, it may rarely accompany inherited immunodeficiency sydromes. The reported childhood cases of PML include three patients, aged 5, 11, and 18 years, with other inherited immunodeficiency syndromes. This patient represents the first time (to our knowledge) that PML has been reported to occur in association with Wiskott-Aldrich syndrome.

Author Affiliations
From the Departments of Neurology (Drs Katz and Berger), Pediatrics (Drs Katz and Hamilton), Internal Medicine (Dr Berger), Microbiology and Immunology (Dr Hamilton), and Radiology (Dr Post), the University of Miami (Fla) School of Medicine; and the Laboratory of Viral and Molecular Pathogenesis (Dr Major), National Institute of Neurological Disorders and Stroke, Bethesda, Md.

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