Sarcoidosis as a cause of transverse myelitis: case report

Abstract

THIS is an unusual case of a patient with paraplegia who had severe neurological and pulmonary involvement and was eventually diagnosed as having sarcoid myelopathy on the basis of the following findings:I. Recurrent neurological problems, including three episodes of acute meningo or encephalo myelitis, immediately preceded by left parotid gland enlargement.2. Parallel with this (but not coinciding with it), recurrent pulmonary problems, including bilateral hilar adenopathy, unexplained cavitation, and recurrent infiltrates.3. Prolonged periods of unexplained fever. Case ReportThis is a case history of a 23-year-old girl, spanning a period of eight years and involving several hospitalisations. The first admission occurred in 1972 for acute meningo myelitis, which rendered the patient paraplegic at T-12 sensory level. The neurological symptoms and signs were preceded by left parotid gland swelling. After two months the patient developed bilateral hilar adenopathy, recurrent pulmonary infiltrates, and at one point, a cavity in the right upper lobe. The pulmonary problems resolved practically without treatment (without antibiotics). A year after the first neurological episode another bout of acute meningo-encephalitis occurred which rendered the patient hemiplegic (left upper extremity and central facial nerve involvement). Without treatment the hemiplegia resolved in two months. During this time the patient had intermittent low grade pyrexia of undetermined aetiology.A third neurological relapse occurred three years later. There was a repetition of an almost explosive onset of aseptic meningitis, immediately followed by tetraplegia. Just prior to this neurological episode the patient had low grade intermittent fever, and swelling of the left parotid gland. At that point, our clinical impression was that she had a systemic disease, possibly sarcoidosis. To confirm our diagnosis of Sarcoid Myelopathy, a Kveim test was performed. Ten days later, the patient developed a nodule at the injection site. This was exactly the time when the patient had her third neurological relapse with quadriplegia. The condition was treated with broad spectrum antibiotics, to which the patient did not respond. Only after steroid treatment did the fever subside and paralysis of both upper extremities and trunk regress completely. Naturally, the nodule which was observed ten days after the Kveim test disappeared completely. Therefore, the biopsy of the nodule was not carried out. 167

Acute Disseminated Encephalomyelitis: Clinical and Pathogenesis Features

“…At the peak of the disease about 50% of patients with ATM are paraplegic; 80% to 94% have been reported to have paresthesia, dysesthesia, or numbness; with almost all the patients suffering from bladder dysfunction [31][32][33][34]. Although ATM can occur in the context of multifocal CNS disease or as a part of multisystemic autoimmune disorders (eg, systemic lupus erythematosus or sarcoidosis) [35,36], it can also present as an isolated idiopathic entity [29]. The initial events leading to detrimental autoimmune responses targeting spinal cord are still a matter of debate, but disease has been pathogenically linked to postinfectious demyelinating disorders [29,37].…”

Neurosarkoidose: Diagnostik und Therapie

“…Da die Radices sehr häufig in einen meningealen Entzündungsprozeß mit einbezogen sind (4, 25,89), können radikuläre Symptome die klinische Symptomatik bestimmen (4). In weniger als 10% der Fälle (107) kann es ohne Therapie zu einer vollständigen Remission kommen (10,26,89,109), Rückfälle sind möglich (10,109).…”