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Encephalomyelitis in primary hypogammaglobulinaemia

P Rudge1, A D Webster, T Revesz

  • 1Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, UK.

Brain : a Journal of Neurology
|February 1, 1996
PubMed
Summary

Primary hypogammaglobulinaemia can cause severe neurological disease, including myelopathy and encephalopathy. Enteroviral infections are a likely cause of these central nervous system complications.

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Area of Science:

  • Neurology
  • Immunology
  • Infectious Disease

Background:

  • Primary hypogammaglobulinaemia, including X-linked agammaglobulinaemia (XLA) and common variable immunodeficiency (CVID), impairs B-cell development and antibody production.
  • Patients with primary hypogammaglobulinaemia are susceptible to recurrent infections, including opportunistic and viral infections.
  • Neurological complications are a significant concern in patients with primary hypogammaglobulinaemia, impacting quality of life and prognosis.

Observation:

  • This study describes the neurological features of 13 patients with primary hypogammaglobulinaemia (7 XLA, 6 CVID).
  • Clinical presentations included progressive myelopathy, myelopathy with encephalopathy, and pure encephalopathy.
  • Additional neurological manifestations observed were retinopathy, sensorineural hearing loss, and dermatomyositis-like features. Cerebrospinal fluid (CSF) analysis revealed pleocytosis in nine patients, and brain imaging showed cerebral atrophy in eight.

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Findings:

  • Enteroviral infection was identified as a probable cause of neurological disease in seven patients, confirmed by PCR or culture.
  • Autopsy findings in four deceased patients revealed end-stage encephalitis, multifocal leukoencephalopathy, and leptomeningitis.
  • Neurological symptoms varied in severity and progression, with some cases of encephalopathy showing temporary reversibility, potentially linked to immunoglobulin therapy.

Implications:

  • Early recognition and diagnosis of neurological complications in primary hypogammaglobulinaemia are crucial for timely intervention.
  • Understanding the role of enteroviral infections can guide diagnostic approaches and potentially inform therapeutic strategies.
  • Further research is needed to elucidate the mechanisms underlying neurological disease in primary hypogammaglobulinaemia and optimize management to improve patient outcomes.