Intracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome: Case report and review of the literature

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Abstract

Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia > 10%, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed.

Original languageEnglish
Pages (from-to)107-111
Number of pages5
JournalJournal of the Neurological Sciences
Volume301
Issue number1-2
DOIs
Publication statusPublished - Feb 15 2011

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Churg-Strauss Syndrome
Cerebral Hemorrhage
Paranasal Sinuses
Asthma
Mononeuropathies
Systemic Vasculitis
Cerebral Angiography
Vascular Malformations
Aphasia
Azathioprine
Eosinophilia
Peripheral Nervous System Diseases
Temporal Lobe
Nose
Eosinophils
Cyclophosphamide
Adrenal Cortex Hormones
Head
Hemorrhage
Drug Therapy

Keywords

  • Central nervous system
  • Cerebral haemorrhage
  • Churg-Strauss syndrome
  • Neurological manifestations
  • Vasculitis

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

Cite this

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title = "Intracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome: Case report and review of the literature",
abstract = "Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia > 10{\%}, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed.",
keywords = "Central nervous system, Cerebral haemorrhage, Churg-Strauss syndrome, Neurological manifestations, Vasculitis",
author = "Mencacci, {Niccol{\`o} E.} and Anna Bersano and Cinnante, {Claudia M.} and Andrea Ciammola and Stefania Corti and Meroni, {Pier Luigi} and Vincenzo Silani",
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T1 - Intracerebral haemorrhage, a possible presentation in Churg-Strauss syndrome

T2 - Case report and review of the literature

AU - Mencacci, Niccolò E.

AU - Bersano, Anna

AU - Cinnante, Claudia M.

AU - Ciammola, Andrea

AU - Corti, Stefania

AU - Meroni, Pier Luigi

AU - Silani, Vincenzo

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N2 - Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia > 10%, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed.

AB - Churg-Strauss syndrome (CSS) is a rare systemic vasculitis, almost invariably accompanied by asthma, nasal polyposis, paranasal sinus abnormalities, and increased peripheral blood eosinophil count. Neurological involvement as peripheral neuropathy is a common feature, whereas cerebral involvement is extremely rare. Herein, we report the case of a young man who presented with sudden onset of right-side emiparesis and aphasia, whose head CT scan showed the presence of large haemorrhage in the left striatum nucleus involving part of the temporal lobe. Based on clinical and laboratory findings (asthma, eosinophilia > 10%, paranasal sinus abnormalities and mononeuritis multiplex) a diagnosis of CSS was made. Cerebral angiography resulted normal, excluding the presence of vascular malformations or signs of vessel abnormalities. Pharmacotherapy with (intravenous and afterwards oral) corticosteroid and immunosuppressors (cyclophosphamide and then azathioprine) was initiated. The outcome was good with neurological follow-up showing a nearly complete recover. Our case points out that intracerebral haemorrhage can be, despite rare, a presenting feature of CSS. Previously reported patients affected by cerebral haemorrhage and CSS are summarized and briefly reviewed.

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