title: Primary cerebral arteritis in a young girl: an unusual cause of acute recurrent neurological deterioration.
abstract: BACKGROUND
  Primary angiitis of the central nervous system (PACNS) is an idiopathic, usually recurrent vasculitis confined to the brain. PACNS has been reported rarely in children, although the disease is probably underdiagnosed.
  
  
  METHODS
  : We report the clinical history of a 3-year-old girl who presented subacute neurological deterioration characterised by headache, speech regression, and altered level of consciousness. Brain MRI revealed severe inflammatory lesions involving both grey and white matters. All blood and cerebrospinal fluid (CSF) tests for inflammatory or infectious processes were negative. Over the next 10 years, the patient relapsed eight times. Brain biopsy confirmed lesions suggestive of cerebral vasculitis. Based on histopathological features and due to the absence of systemic vasculitis, the patient was considered to have PACNS. She developed partial epilepsy, and clinical stabilisation was finally achieved via continuous oral corticosteroids and immunosuppressive agents.
  
  
  CONCLUSIONS
  PACNS may be the cause of subacute and relapsing inflammatory encephalopathy in children after excluding other diagnoses, such as multiple sclerosis, sarcoidosis, recurrent acute disseminated encephalomyelitis (ADEM), and primary central nervous system lymphoma. Brain biopsy is necessary to confirm the diagnosis of PACNS and exclude diseases with similar symptoms. Neurological outcome remains poor.
authors.affiliation: Université catholique de Louvain, Cliniques universitaires Saint-Luc, avenue Hippocrate 10, B-1200 Bruxelles, Belgium.
authors.forename: Bouchra
authors.initials: B
authors.lastname: El M'Kaddem
authors.forename: Philippe
authors.initials: P
authors.lastname: Clapuyt
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: de Bruycker
authors.forename: Bernard
authors.initials: B
authors.lastname: Lauwerys
authors.forename: Christian
authors.initials: C
authors.lastname: Raftopoulos
authors.forename: Catherine
authors.initials: C
authors.lastname: Godfraind
authors.forename: Marie-Cécile
authors.initials: MC
authors.lastname: Nassogne
mesh_terms: D000208:Acute Disease; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001259:Ataxia / Q000209:etiology; D001379:Azathioprine / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D002536:Cerebral Arteries / Q000473:pathology; D002675:Child, Preschool; D060825:Cognitive Dysfunction / Q000209:etiology / Q000523:psychology; D003520:Cyclophosphamide / Q000627:therapeutic use; D006970:Disorders of Excessive Somnolence / Q000209:etiology; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D019636:Neurodegenerative Diseases / Q000209:etiology*; D012008:Recurrence; D000069283:Rituximab; D013064:Speech Disorders / Q000209:etiology; D013256:Steroids / Q000627:therapeutic use; D020293:Vasculitis, Central Nervous System / Q000150:complications*; D014888:Wechsler Scales
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D013256:Steroids; D000069283:Rituximab; D003520:Cyclophosphamide; D001379:Azathioprine