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Neurosarcoidosis: Clinical Features, Pathogenesis, and Management

Ragav Aachi, Marjorie Fowler, Eduardo Gonzalez-Toledo, Jeanie McGee, and Alireza Minagar

Introduction

Sarcoidosis is an idiopathic, multi-organ, immune-mediated, inflammatory disorder of unrecognized cure characterized by the development of non-caseating epithelioid granulomas. As a systemic disorder, it heavily involves the respiratory and lymphatic systems (particularly intrathoracic lymph nodes) as well as the skin. Nervous system involvement in the course of sarcoidosis (neurosarcoidosis) is uncommon and occurs only in 5-10 % of cases. Interestingly, neurologic symptoms can be the only presentation of sarcoidosis in 10-17 % of individuals [1]. Despite these figures, the exact prevalence of nervous system involvement in the course of sarcoidosis is believed to be higher since subclinical involvement of the nervous system has been reported in up to 27 % of patients with sarcoidosis on autopsy [2]. Neurosarcoidosis comprises a wide gamut of clinical presentations which stem from involvement of both central and peripheral components of the human nervous system; therefore, it can imitate many other neuropathologies. Clinically, neurosarcoidosis presents with cranial nerve(s) involvement (facial nerve palsy is particularly common), aseptic meningitis, diencephalic syndromes (particularly hypopituitarism), epilepsy, cognitive decline, myelopathy, and peripheral neuropathy.

R. Aachi, MD • J. McGee, DHEd, MSHS • A. Minagar, MD (*)

Department of Neurology, LSU Health Sciences Center,

1501 Kings Highway, Shreveport, LA 71130, USA e-mail: This email address is being protected from spam bots, you need Javascript enabled to view it

M. Fowler, MD

Departments of Pathology, LSU Health Sciences Center, Shreveport, LA 71130, USA E. Gonzalez-Toledo, MD, PhD

Department of Neurology, LSU Health Sciences Center,

1501 Kings Highway, Shreveport, LA 71130, USA

Departments of Radiology, LSU Health Sciences Center, Shreveport, LA 71130, USA © Springer International Publishing AG 2017

A. Minagar, J.S. Alexander (eds.), Inflammatory Disorders of the Nervous System, Current Clinical Neurology, DOI 10.1007/978-3-319-51220-4_5

x4XX: Low-power photomicrograph showing granulomas within the meninges. A small granuloma is seen in the parenchyma of the brain (H&E, original magnification x 40)

Fig. 5.1 10x4XX: Low-power photomicrograph showing granulomas within the meninges. A small granuloma is seen in the parenchyma of the brain (H&E, original magnification x 40)

Neuropathologically, sarcoidosis is characterized by the presence of epithelioid, non-caseating granulomas, including clusters of activated and highly differentiated macrophages and other epithelioid cells surrounded by T lymphocytes (Figs. 5.1 and 5.2). Langerhans-type multinucleated giant cells are commonly present, and further examination of the granulomas reveal that the center consists mainly of CD4+ lymphocytes, while the CD8+ lymphocytes exist in the periphery (Figs. 5.1, 5.2, and 5.3). Also, tumor necrosis factor-а (TNFa) is implicated in the pathogenesis of nervous system inflammation [3].

 
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