Common variable immunodeficiency with coexisting central nervous system sarcoidosis. Case report and literature review with implications for diagnosis and pathogenesis

Magdalena Dziadzio, T. Hortobágyi, Desmond Kidd, Ronnie Chee

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

We describe a patient with a history of longstanding primary generalised epilepsy, on anticonvulsant therapy, who presented with fever, headache, worsening seizures and hallucinations. Among various investigations, the patient had high CSF protein and ACE levels, leptomeningeal nodular enhancement on MRI brain and non-caseating granulomas in the brain and meninges on the biopsy. The patient was diagnosed with neurosarcoidosis. Subsequently, he was found to be panhypogammaglobulinaemic and was diagnosed with probable common variable immunodeficiency (CVID). The coexistence of common variable immunodeficiency and neurosarcoidosis is rare. Typically, non-caseating granulomas in CVID patients are localised in the lymphatic tissue and solid organs. To our knowledge, there are only five reports of the granulomas of the central nervous system (CNS) in CVID. We discuss the diagnostic difficulties in this case and review the literature.

Original languageEnglish
Pages (from-to)405-408
Number of pages4
JournalIdeggyógyászati szemle
Volume64
Issue number11-12
Publication statusPublished - Nov 30 2011

Fingerprint

Common Variable Immunodeficiency
Central Nervous System
Granuloma
Generalized Epilepsy
Meninges
Hallucinations
Brain
Lymphoid Tissue
Anticonvulsants
Headache
Seizures
Fever
Biopsy
Neurosarcoidosis
Proteins

Keywords

  • Anticonvulsants
  • Common variable immunodeficiency
  • Granuloma
  • Hypogammaglobulinaemia
  • Leptomeningeal enhancement
  • Neurosarcoidosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

Common variable immunodeficiency with coexisting central nervous system sarcoidosis. Case report and literature review with implications for diagnosis and pathogenesis. / Dziadzio, Magdalena; Hortobágyi, T.; Kidd, Desmond; Chee, Ronnie.

In: Ideggyógyászati szemle, Vol. 64, No. 11-12, 30.11.2011, p. 405-408.

Research output: Contribution to journalArticle

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