Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2014, 158(4):662-667 | DOI: 10.5507/bp.2013.047

Neurosarcoidosis: Two case reports with multiple cranial nerve involvement and review of the literature

Milija Mijajlovic^a, Mihailo Mirkovic^b, Violeta Mihailovic-Vucinic^c, Vuk Aleksic^a, Nadezda Covickovic-Sternic^a

^a Neurology Clinic, Clinical Center of Serbia and School of Medicine, University of Belgrade, Serbia

^b Department of Neurology, Health Center Valjevo, Valjevo, Serbia

^c Clinic for Pulmonary Diseases and Tuberculosis, Clinical Center of Serbia and School of Medicine, University of Belgrade, Serbia

Background: Involvement of the central nervous system is registered in a relatively small number of patients with sarcoidosis. In this article we present two cases with various neurological symptoms that fulfill criteria for neurosarcoidosis (NS). In addition, we review the literature on NS with special attention to isolated cranial nerve involvement.

Methods and Results: First patient: Neurological examination identified multiple cranial neuropathy, moderate right-sided hemiparesis, polyradiculoneuritis of the lower limbs and positive meningeal signs. Laboratory tests showed serum and cerebrospinal fluid (CSF) inflammatory abnormalities, with increased values of the angiotensin-converting enzyme (ACE). CSF analysis also showed presence of 9 oligoclonal IgG bands. Brain and spine magnetic resonance imaging (MRI) revealed diffuse meningopathy, and focal granulomatous lesion in the body of the L5 vertebra. Lung sarcoidosis was confirmed by additional diagnostic procedures. The patient was treated with Methylprednisolone and a tapering course of oral Prednisone, which reduced the pain in the back and legs and improved the strength of the right leg. However, the other neurological deficiencies remained. After confirming lung sarcoidosis, the patient received Methotrexate in addition to Prednisone but during the following 2 years the patient's condition progressively worsened and ended in death.

Second patient: Neurological findings showed weakness of the right n. oculomotorius and the right n. trochlearis, as well as the right-side face weakness. We found raised level of the ACE in serum and CSF. Thorax high-definition computed tomography (HDCTT) showed ribbon-like domains of discrete changes in the pulmonary parenchyma. MRI of the brain showed multiple white matter lesions. This patient also received Methylprednisolone followed by Prednisone, and after two months, ocular motility normalized.

Conclusion: The diagnosis of NS is always a challenge. For this rerason definitive diagnosis requires the exclusion of other causes of neuropathy. Multiple cranial neuropathies should always arouse suspicion of NS.

Keywords: sarcoidosis, neurosarcoidosis, multiple cranial neuropathies

Received: November 24, 2012; Accepted: June 6, 2013; Prepublished online: June 27, 2013; Published: December 9, 2014  Show citation