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Relapsing encephalopathy with headache: an unusual presentation of isolated intracranial neurosarcoidosis
  1. Adam K Rudkin1,
  2. Robert A Wilcox1,
  3. Mark Slee1,
  4. Anne Kupa2,
  5. Dominic Thyagarajan3
  1. 1Department of Neurology, Flinders Medical Centre, Adelaide, Australia
  2. 2Department of Immunology, Allergy and Arthritis, Flinders Medical Centre, Adelaide, Australia
  3. 3Department of Neurology, Flinders Medical Centre, Adelaide, Australia
  1. Correspondence to:
 Robert A Wilcox
 Department of Neurology, Level 2, Flinders Medical Centre, Bedford Park, Adelaide, 5042, Australia; Robert.Wilcox{at}fmc.sa.gov.au

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We report a presentation of relapsing and remitting isolated intracranial neurosarcoidosis in a female patient who presented with episodic severe headache and behavioural disturbance initially misdiagnosed as psychosis. Eventually, several episodes were accompanied by visual disturbance secondary to papilloedema, ultimately leading to a diagnosis of neurosarcoidosis on meningeal biopsy.

Sarcoidosis is a multisystem inflammatory disease of unknown aetiology, and is characterised by non-caseating granulomata. Pulmonary disease is the most common manifestation, occurring in 90% of patients. Clinical involvement of the nervous system is said to occur in 5–15% of patients.1 Isolated intracranial neurosarcoidosis is even rarer, with systemic sarcoidosis being detected in more than 95% of cases of sarcoidosis initially presenting with neurological symptoms.2

Case report

A woman presented initially at age 30 years, and then subsequently five times over 3 years with stereotyped episodes of headache, confusion and psychomotor agitation. In each instance, a history was given of a constant, severe bifrontal headache that was associated with nausea, vomiting, photophobia and/or visual field disturbance. As each presentation evolved, she became confused and encephalopathic. Early in the course of her illness these episodes were misinterpreted as acute psychosis because she exhibited pressured …

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