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Indexed/Abstracted in: e-psyche, EMBASE, PubMed/MEDLINE, Neuroscience Citation Index, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,651
Online ISSN 1827-1855
Miscusi M., Polli F. M., Missori P., Delfini R.
Department of Neurological Sciences Neurosurgery, University of Rome ''La Sapienza'', Italy
We present a case of isolated cerebral neurosarcoidosis with remitting nodular lesions resembling intraventricular neoplasms. The patient, admitted at our Department for surgical treatment of a magnetic resonance imaging (MRI) demonstrated fourth ventricle lesion, presented acute intracranial hypertension. A second MRI performed before the scheduled operation showed the disappearance of the fourth ventricle lesion and a tetra-ventricular hydrocephalus. The patient has been treated with a third-ventriculostomy, followed, after 15 days, by ventriculoperitoneal shunt. High doses of steroids have been administered. Cerebrospinal fluid analysis has been conducted and a high concentration of ACE, specific marker of neurosarcoidosis, has been found. No biopsy was performed to avoid surgical complications. A systemic chemotherapy with azathioprine has been started, but the patient died six months later for worsening of the clinical conditions. The autopsy confirmed the diagnosis of neurosarcoidosis. This is the first MRI documented case of neurosarcoidosis with remitting lesions. Diagnosis of isolated neurosarcoidosis is difficult and it is based on clinical and radiological exclusion of other entities. CSF examination is useful for diagnosis, for the reported specificity and sensibility of ACE. Medical treatment is based on corticosteroids and chemotherapic agents. Neurosurgical intervention can be related to treat hydrocephalus due to ependymal and arachnoidal involvement or to remove large lesions. In our opinion biopsy should be limited only to ACE negative patients and to those who do not respond to chemotherapy.