Dados do Trabalho

Título

Neurosarcoidosis presenting as “acute” normal pressure hydrocephalus: a case report

RESUMO

Case presentation: A 68-year-old woman, with a previous medical history of diabetes, hypertension and hypothyroidism, was brought to the emergency department with complaints of bilateral leg weakness, cognitive impairment and urinary incontinence that started twenty days prior to admission. The patient also reported unmeasured daily fever over the preceding two weeks, a significant unintentional weight loss and anosmia over the past two years. At the initial evaluation, a mild loss of strength in the lower limbs and significant gait apraxia were observed. Initial head CT showed dilatation of supratentorial ventricular system and transependymal effusion of cerebrospinal fluid (CSF). CSF analysis demonstrated a cellularity of 12 leukocytes/mm3 (100% lymphocytes), increased total protein (63mg/dL) and a low glucose level (CSF/plasma ratio of 52%). The MRI showed signs of leptomeningeal and choroid plexus inflammation, with obstructive hydrocephalus and intracranial hypertension. Following the investigation, a chest CT demonstrated paratracheal and subcarinal lymphadenopathy and a biopsy was performed. The fungal, bacterial and mycobacterial cultures of CSF and lymph node were negative and pathologic examination of the lymph node revealed noncaseating granulomas without evidence of malignancy. A diagnosis of neurosarcoidosis with obstructive hydrocephalus was made and the patient was started on steroids (1mg/Kg/day of prednisone). After the beginning of therapy, the patient presented significant improvement of the symptoms, returned to walk independently and there was no need of neurosurgical procedures to drain the CSF.

Discussion: Sarcoidosis is a multisystemic granulomatous disorder characterized by the formation of noncaseating granuloma in various organ systems. Neurological involvement may result in peripheral or central nervous system (CNS) manifestations. Gait disorders and cognitive decline are less common forms of presentation. Here, we report an unusual presentation of the disease. A patient with a previously preserved functionality presented a rapidly progressive cognitive impairment, gait apraxia and urinary incontinence. Given the challenging diagnosis of the case and association with obstructive hydrocephalus, clinicians should be aware of this condition.

Final comments: The case presented demonstrates an unusual association of neurosarcoidosis and a rapidly progressive presentation of cognitive impairment, gait apraxia and urinary incontinence.

Palavras Chave

Neurosarcoidosis, Obstructive hydrocephalus, Gait apraxia