Dados do Trabalho

Título

Aseptic meningoencephalitis and peripheral neuropathy - a rare association of neurosarcoidosis

RESUMO

Case Presentation
A 67-year-old female, began symptoms of muscular weakness on both legs in June 2021 that evolved to an ataxia of gait on a two months period. At hospital admission, she had sensitive ataxia, hyporreflexia and mild weakness on lower limbs. There were neither cranial nerve palsies nor other abnormalities. After five days of hospitalization, she presented generalized seizures and stupor, requiring mechanical ventilatory support. The liquor was clear with an open pressure of 15 cmH20, a protein level of 150 mg/dl, 74 mg/dl Glucose, and 14/mm³ white cell count (78% lymphocytes, 22% monocytes); VDRL, test for M. tuberculosis and PCR for multiple viruses were all negative and no bacteria or fungus were isolated on cultures. Brain and spinal cord MRI were normal. Chest Computed Tomography showed bilateral hilar and paratracheal adenomegaly.There was non-necrotizing granulomatous lymphadenitis on lymph node biopsia. These elements allowed us to diagnose neurosarcoidosis (NS). She treated with methylprednisolone 1g/day i.v. for 5 days followed by oral prednisone 5 mg/day until discharge and two antiseiuzure drugs for epilepsy control. Neurological improvement was achieved along the first week with the patient going out the ventilator and becoming ambulatory with almost normal gait after thirty days. Homewer, she developed osteomyelitis and died after clinical complications.
Discussion
Sarcoidosis is a multisystem inflammatory disorder characterized by noncaseating granulomas in various organ systems, mainly the lungs and the lymphatic system. NS occurs in about 5–10% of patients with sarcoidosis and 50% of patients suspected of NS presents neurologic symptoms as first manifestation. Cranial neuropathy is the most common manifestation (mainly facial nerves) and is seen in 50–70% of cases. Peripheral nerve involvement occurs in 10% and brain and spinal cord lesions are less prevalent. Here we report an even rarer presentation of NS: aseptic meningitis of chronic evolution associated with peripheral neuropathy. Glucocorticoids are considered to be first-line therapy. It is associated with good efficacy and rapid clinical response. Severe presentations may require pulse-dose IV methylprednisolone, followed by a prolonged oral glucocorticoid tapering.
Final Comments
This case illustrates a rare presentation of neurosarcoidosis that deserves adequate recognition and prompt treatment to avoid neurological sequelae or even the patient's death.

Palavras Chave

neurosarcoidosis, Aseptic meningoencephalitis, peripheral neuropathy