Sarcoidosis Presenting as an Intramedullary Spinal Cord Lesion

Abstract

ABSTRACT:Objectives:Meige syndrome is a movement disorder that includes blepharospasm and oromandibular dystonias. Its etiology may be idiopathic (primary) or it may arise secondary to focal brain injury. Acute respiratory distress as a feature of such dystonias occurs infrequently. A review of the literature on Meige syndrome and the relationship between dystonias and respiratory compromise is presented.Methods:A 60-year-old woman suffered a cerebral anoxic event secondary to manual strangulation. She developed progressive blepharospasm combined with oromandibular and cervical dystonias. Neuroimaging demonstrated bilateral damage localized to the globus pallidus. Years later, she presented to the emergency department in intermittent respiratory distress associated with facial and cervical muscle spasms.Results and conclusions:Increasing frequency and severity of the disorder was noted over years. The acute onset of respiratory involvement required intubation and eventual tracheotomy. A partial therapeutic benefit of tetrabenazine was demonstrated.

Isolated spinal neurosarcoidosis: An enigmatic intramedullary spinal cord pathology-case report and review of the literature

“…[4562839] Isolated NS is uncommon; with that restricted to the spinal cord being exceptionally rare. [91112131415161718192021222324252627] When involved, the cervical, thoracic, and lumbar spinal cord is involved in that sequence. Spinal sarcoidosis can present with varied symptoms, including weakness; paresthesias; myelopathy; demyelinating syndrome; facial nerve paralysis; bowel, bladder, or sexual disturbances; and back pain or even radicular pain which are no different from other spinal cord pathologies which are much more common than spinal sarcoidosis.…”

“…[45678910] This however is not always true and sarcoidosis albeit rarely; can manifest as true isolated spinal cord involvement in less than 0.5% cases. [91112131415161718192021222324252627] The rarity of this presentation with nondiagnostic imaging features, clinical presentation, and absence of systemic involvement often makes it challenging to diagnose this condition leading to delay in diagnosis and often inappropriate management with concomitant morbidity. [23] Blood and cerebrospinal fluid (CSF) markers are mostly adjunct and have very low sensitivity and specificity for NS confounding the diagnosis.…”

“…[3132] Though these cases initially presented with features of spinal cord involvement, workup revealed presence of extraneural involvement,[313233] as against very few cases described in the literature wherein true isolated spinal cord NS was described and mandated biopsy. [2911121415161718202122232425262731333435363738] We encountered a case of isolated cervical intramedullary NS which presented as cervical myelopathy and could be diagnosed only after performance of spinal cord biopsy meeting the definite Zagilek's criteria. The clinical picture with nuances of diagnosis of this condition from the present case is discussed with comprehensive review of the literature and characteristic MRI features of spinal NS which might be useful for diagnosis/workup.…”

Isolated Spinal Cord Neurosarcoidosis Diagnosed by Cord Biopsy and Thalidomide Trial

“…This is a rare case confirmed by direct tissue biopsy in the spinal cord. A few cases with neurosarcoidosis have been reported, however most cases suggested only clinical manifestation without histology, or demonstrated indirect biopsy in the brain, meninges and lymph node (6-10). …”

“…In general, patients with spinal sarcoidosis are considered to be at high risk for severe neurological sequelae (7). The information available about spinal sarcoidosis management and diagnosis comes from a few case reports, small series and expert opinions (8-10). However, the documents gave conflicting conclusions regarding the treatment, including corticosteroids and alternative immunosuppressants (6, 7).…”