Neurosarcoid Presents Differently in Children Than in Adults

Baumann, Robert J.; Robertson, William C.

doi:10.1542/peds.112.6.e480

Cited by 88 publications

(57 citation statements)

References 35 publications

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“…Large-vessel vasculitis associated with abdominal aortic aneurysm which has previously been reported by our group [13] was seen in a case with EOCS. CNS disease, although common in adults, is rare in children [14]. In the present report, involvement of the central nervous system (CNS) manifested in two patients.…”

Section: Discussionmentioning

confidence: 65%

Childhood sarcoidosis: Louisiana experience

et al. 2015

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A retrospective chart review was conducted to detect patients with sarcoidosis seen by pediatric rheumatology service from the period of 1992 to 2013 at Children's hospital of New Orleans. Twenty-seven patients were identified. The average duration of symptoms before diagnosis was 5 (range 1-120) months. Five patients had onset before the age of 5 years and were diagnosed with early-onset sarcoidosis. The most common manifestations at presentation were constitutional symptoms (62 %) followed by ocular (38 %). During the course of illness, 19/27 (70 %) had multiorgan involvement. Common manifestations included uveitis/iritis (77 %), fever (50 %), hilar adenopathy (42 %), arthritis (31 %), peripheral lympadenopathy (31 %), hepatosplenomegaly (31 %), parenchymal lung disease (27 %), and skin rash (19 %). Unusual manifestations included granulomatous bone marrow disease (3 cases), hypertension (2), abdominal aortic aneurysm (large vessel vasculitis; 1), granulomatous hepatitis (1), nephrocalcinosis (1), membranous nephropathy (1), refractory granulomatous interstitial nephritis with recurrence in transplanted kidney (1), CNS involvement (2), parotid gland enlargement (1), and sensorineural hearing loss (1). Biopsy specimen was obtained in 21/27 (77 %) patients, and demonstration of noncaseating granuloma associated with negative stains for mycobacteria and fungi was seen in 18 patients. Elevated angiotensin-converting enzyme level was seen in 74 % of patients. Treatment with oral prednisone was initiated in symptomatic patients with significant clinical improvement. Low-dose methotrexate (MTX) 10-15 mg/m(2)/week orally, as steroid-sparing agent, was administered in 14 patients. Other immunomodulators included cyclophosphamide (2 patients), etanercept (2), infliximab (2), mycophenolate mofetil (1), and tacrolimus (1). Childhood sarcoidosis is prevalent in Louisiana. Most of the affected children present with a multisystem disease associated with manifestations similar to those of adult patients. Low-dose MTX seems to be effective, steroid sparing, and safe adjunct to treat sarcoidosis with multiorgan involvement. Early-onset disease is less common and associated with increased morbidity, flares, and poor prognosis.

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Section: Discussionmentioning

confidence: 65%

Childhood sarcoidosis: Louisiana experience

et al. 2015

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“…Manifestations of neurosarcoidosis include cranial nerve palsies, meningeal involvement, brain lesions, seizures, hypothalamic and endocrine dysfunction, and peripheral neuropathy 13 . Unlike adults, who characteristically present with a seventh cranial nerve palsy, prepubertal children are more likely to present with seizures and are perhaps more likely to have a space-occupying lesion 6 . Twenty nine cases of childhood neurosarcoidosis have been reported, with 38% (11/29) presenting with seizures and 24% (7/29) with mass lesions or focal edema on imaging.…”

Section: Discussionmentioning

confidence: 99%

“…Twenty nine cases of childhood neurosarcoidosis have been reported, with 38% (11/29) presenting with seizures and 24% (7/29) with mass lesions or focal edema on imaging. Of the seven that presented with mass lesions or focal edema on imaging, three patients presented with seizures 6 . Neurosarcoidosis may remit spontaneously 7 .…”

Section: Discussionmentioning

confidence: 99%

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

Scholten

Hove²,

MacDonald³

2009

Can. j. neurol. sci.

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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

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“…Seizures occur in 38% of children with CNS sarcoidosis while 21% have cranial nerve involvement, most often facial nerve palsy [16]. Other manifestations of CNS sarcoidosis may include hypothalamic dysfunction, headache, or mass lesion.…”

Section: Discussionmentioning

confidence: 99%

Sarcoidosis presenting with hearing loss and granulomatous interstitial nephritis in an adolescent

et al. 2006

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Granulomatous interstitial nephritis is an uncommon finding in a kidney biopsy. The differential diagnosis is broad and includes infections, drug exposure, and sarcoidosis. Sarcoidosis, a systemic disorder of unknown etiology characterized by the presence of noncaseating granulomata in affected organs, is rare in children. We discuss an adolescent boy with the unusual presentation of granulomatous interstitial nephritis and acute deafness. Sarcoidosis should be considered as part of the differential diagnosis for children and adolescents with hearing loss and kidney disease.

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Neurosarcoid Presents Differently in Children Than in Adults

Cited by 88 publications

References 35 publications

Childhood sarcoidosis: Louisiana experience

Childhood sarcoidosis: Louisiana experience

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

Sarcoidosis presenting with hearing loss and granulomatous interstitial nephritis in an adolescent

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