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

Abstract

aBackground. 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 rightsided 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.

Neuroprotective effect of lurasidone via antagonist activities on histamine in a rat model of cranial nerve involvement

“…Several potentially neuroprotective agents function to upregulate gene transcription at the onset of brain ischemia, which may be an effective approach for limiting brain tissue damage (25). Studies on the mechanisms of cranial nerve involvement have shown that the serotonin systematic signaling pathway shows high correlation with the initiation, development, treatment and prognosis of cranial nerve involvement (26,27). In addition, an increasing number of studies have reported that the overexpression of histamine in the cerebrospinal fluid is a potential therapeutic target in patients with cranial nerve involvement and has been found be important in the pathogenesis of cranial nerve involvement (28)(29)(30)(31).…”

An atypical case of neurosarcoidosis presenting with neovascular glaucoma

“…Involvement of the CNS, referred to as neurosarcoidosis (NS), occurs in only 5–15% of cases [ 14 , 22 ] with cranial neuropathies as the most frequent neurological manifestation [ 12 , 23 ]. Neuro-ophthalmic sarcoidosis is a specific subset of NS of unknown frequency that involves the afferent and efferent visual systems [ 11 ].…”

Vertigo as a Predominant Manifestation of Neurosarcoidosis

“…Lumbar puncture in sarcoidosis may demonstrate an elevated CSF protein and a mild-to-moderate pleocytosis with lymphocytic predominance or may be normal in a third of patients [ 9 , 10 ]. Decreased glucose levels may occur in one-fifth of patients [ 10 ]. Our patient's CSF showed lymphocytic predominance with normal protein.…”