An Unusual Presentation of Neurosarcoidosis in an 11-Year-Old Boy

Abstract

Sarcoidosis is a chronic, multisystem, granulomatous disease which typically develops between the ages of 20 and 40 years 1 . Neurosarcoidosis occurs in only 5-15% of adults with sarcoidosis, and is seldom reported in children [2][3][4][5] . Of the cases described in the literature, children were found more likely to present with seizures, and less commonly space-occupying lesions 6 . The tumefactive brain lesions are difficult to distinguish from the tumefactive demyelinating lesions of multiple sclerosis, from brain neoplasms, acute disseminated encephalo-myelitis (ADEM), or from parasitic foci 7,8 . We report the clinical and radiographic features of an 11-year-old boy with biopsy-proven neurosarcoidosis in order to highlight key features that distinguish neurosarcoidosis from tumefactive demyelination. CASE REPORTA previously healthy 11-year-old male presented in status epilepticus. The seizure started with an altered level of awareness and focal twitching of the right side of his face. The ictus lasted 45 minutes and responded to intravenous administration of lorazepam and phenytoin.Investigations revealed a peripheral white blood cell count of 28.2 (4.5-13 X10 9 /L), and normal hemoglobin and platelets. Electrolytes, glucose, renal and liver function, and coagulation parameters were normal. A toxicology screen was negative. Bacterial, viral, and fungal cultures were negative in blood. Cerebrospinal fluid (CSF) analyses revealed 20 X 10 6 /L leucocytes (90% lymphocytes), normal glucose and protein, no malignant cells, and negative bacterial and viral cultures. Polymerase chain reactivity for Herpes viruses was negative. Oligoclonal banding was detected, but as serum electrophoresis was not performed, this finding could not be evaluated. Serum anti-nuclear factor was negative.Computerized tomography of the head showed a focal, nonenhancing mass of the left frontal lobe with no evidence of mass effect or hydrocephalus. As shown in Figure 1, magnetic resonance imaging (MRI) demonstrated a region of increased T2/FLAIR signal involving the white matter of the left frontal lobe with leptomeningeal enhancement after gadolinium contrast administration. Magnetic resonance spectroscopy (MRS) demonstrated a lactate peak with normal choline and Nacetylaspartate. A second MRI obtained one week later showed improvement, with normalization of mass spectroscopy.Following recovery from the post-ictal period, the child was noted to have a normal neurological and general physical examination, including normal cognition and no focal deficits. Given the normal examination, and repeat MRI showing improvement in the lesion appearance, brain biopsy was THE CANADIAN JOURNAL OF NEUROLOGICAL SCIENCES 783

Clinical and radiological features of pituitary stalk lesions in children and adolescents

“…It occurs throughout the world in all races, and the highest annual incidence of 5 to 40 cases per 100,000 people has been observed in northern European countries 12 , 13) . Childhood-onset sarcoidosis is rare and can occur as a part of systemic sarcoidosis or as an isolated presentation 9 , 14 , 15 , 16) .…”

Masquerades of Acquired Demyelination in Children

“…Features of this 11-year-old boy are available in a previous publication. 7 Briefly, this previously healthy child presented with status epilepticus. MRI demonstrated a region of increased T2 signal involving the white matter of the left frontal lobe with focal leptomeningeal enhancement.…”

Demyelinating CNS Lesions in a Teenager

“…Isolated neurosarcoidosis is very rare, with >95% of patients exhibiting systemic sarcoidosis despite presenting initially with neurological symptoms. 9 Our patient exhibited low ACE serology and normal chest CT, spinal MRI, and CSF studies, which argues against this diagnosis.…”