Abstract Details

Title The Clinical Heterogeneity of Neurosarcoidosis: A Comprehensive Study of 132 Patients

Topic MS and Related Diseases

Presentation(s) P02 - (-)

Poster/Presentation
Number 116

Objective

Background BACKGROUND: Sarcoidosis is a systemic granulomatous disorder that manifests with pulmonary and lymph node involvement. The diagnostic of neurosarcoidosis presents a challenge due to its diversity in clinical presentations and its mimicry with other central nervous system (CNS) disorders.

Design/Methods DESIGN/METHODS: Approximately 300 patients were evaluated at the Johns Hopkins Neuroimmunology clinic from January 2000 - August 2012 for the presumptive diagnosis of Neurosarcoidosis The clinical features as well as neuroimaging, CSF, and biopsy findings were assessed to establish the profiles of neurological involvement.

Results RESULTS: 132 patients met the criteria for diagnosis of definite, probable or possible neurosarcoidosis based on clinical, imaging and biopsy data. In 60% (81) of the patients, the neurological symptoms were the first manifestation of the disease. Definite diagnosis of neurosarcoidosis was established in 33 patients after a brain, meningeal, cord, nerve or muscle biopsy while the diagnosis of probable neurosarcoidosis was supported by neurological manifestations in 89 patients who had positive non-CNS tissue biopsy for sarcoidosis. We identified 6 major neurological presentations in neurosarcoidosis: Meningeal (n=58), Encephalitic (n=55), Myelopathic (n=49), Cranial Neuropathy (n=37), Neuroendocrine (n=10), and Neuromuscular (n=5). 68 patients (51%) presented overlap profiles in which more than one neurologic form was present, including 17 (13%) patients that exhibited widespread involvement. CSF studies performed during the acute stage in 51 patients showed pleocytosis and increased proteins in 76% and 70% of the patients respectively.

Conclusions CONCLUSIONS: Neurological forms of sarcoidosis exhibit a heterogeneous clinical features and variable temporal profiles. Although the most common presentation of this pathology is pulmonary sarcoidosis, neurological symptoms may represent the first manifestation of the disease in more than half of the patients with neurosarcoidosis.

Authors/Disclosures
Maria I. Reyes-Mantilla, MD (Johns Hopkins University, Neurology) PRESENTER	Dr. Reyes-Mantilla has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Carlos A. Pardo-Villamizar, MD (Johns Hopkins U, Med Dept of Neurology)	The institution of Dr. Pardo-Villamizar has received research support from National Institutes of Health. The institution of Dr. Pardo-Villamizar has received research support from Bart McLean Fund for Neuroimmunology Research .
Eelco F. Wijdicks, MD, FAAN (Mayo Clinic W8)	Dr. Wijdicks has received publishing royalties from a publication relating to health care.
Eelco F. Wijdicks, MD, FAAN (Mayo Clinic W8)	Dr. Wijdicks has received publishing royalties from a publication relating to health care.

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