Central Nervous System Manifestations of Sarcoidosis in Children

Abstract

We report a case of central nervous system (CNS) sarcoidosis in a young girl and review the literature on the CNS manifestations of sarcoidosis in 23 cases occurring in children.NUMEROUS extensive reviews of sarcoidosis in the pediatric age group have been published.'-' The neurologic manifestations of sarcoidosis have also been extensively reviewed.9-16 However, the unusual occurrence of central nervous system (CNS) sarcoidosis in children has not been reviewed previously. We present a case report as an introduction to a literature survey. Case ReportA 13-year-old black girl came to The Brookdale Hospital Medical Center with a one-month history of fever, malaise, arthralgias, vomiting, and weight loss. Several days prior to admission, she complained of blurred vision. The temperature was 38.5 C, pulse 80/min, respiratory rate 24/ min, and blood pressure 120/80 mm Hg. Her skin revealed hyperpigmented confluent lesions on the extensor surface of arms and legs. Results of examination of head, ears, nose, and throat were within normal limits. Her sclerae were injected, and she was photophobic. A slit-lamp examination latter revealed a granulomatous panuveitis. No lymphadenopathy was noted. Examination of her heart and lungs was unremarkable. Her spleen was not palpable but her liver was enlarged 4 cm below the right costal margin. The result of admission neurologic examination was normal. Initial laboratory values were WBC count 8800 with 30% bands, 34% segmented neutrophils, 26% lymphocytes, and 7% eosinophils; hemoglobin 11.0 g; platelets 686,000; and a sedimentation rate of 64 mm/hr; PT 13/13; PTT 44 sec; SGOT 48 U/L; SGPT 29 U/L; CPK 179 U/L; LDH 48-2 U/L; cholesterol 133 mg/dl; calcium 8.8 mg/ dl; phosphorus 2.8 mg/dl; uric acid 4.9 mg/dl; BUN 12 mg/dl; glucose 80 mg/dl; creatinine 1.2 mg/dl; alkaline phosphatase 262 IU; sodium 132 mEq/L, potassium 3.4 mEq/L, chloride 95 mEq/L, CCI~ 25 mEq/L. Her urine was 1 + for protein with 3-5 WBC/hpf. Results of latex fixation and screening anti-nuclear antibody tests were negative. A normal chest x-ray and liver scan were also obtained. Pulmonary function testing revealed a severely reduced vital capacity of 30 per cent of predicted value. Diffusion capacity by steady state method was also 30 per cent of predicted. Systemic sarcoidosis was suspected but a conjunctival and liver biopsy were negative.With an empirical diagnosis of sarcoidosis, treatment was begun with systemic therapy of 40 mg of prednisone daily, and she was discharged. At home she continued to have nausea, anorexia, weight loss, headache, and arthralgia. Two weeks later she was readmitted with head nodding and persistent twitching of the right side of her face and right arm. An electrc~encephalogram showed sharp wave build-up in the left midtemporal area leading to generalized seizure activity. A CT scan was normal. Lumbar puncture revealed I WBC/hpf with a CSF protein of 49 mg/l. The diagnosis of systemic sarcoidosis was confirmed by skin biopsy (Fig. 1) which showed a noncaseating granuloma. No

Overview and report on international registry of sarcoid arthritis in childhood

“…Neurologic manifestations are quite varied and include mass lesions, cranial nerve involvement, seizure disorders, encephalopathy, aseptic meningitis, myelopathy, and spinal cord involvement [4,23,24]. Six of 22 registry patients had evidence of neurologic involvement.…”

Neurosarcoid Presents Differently in Children Than in Adults

“…An additional 15 cases lacked sufficient data about presenting signs and symptoms and were omitted. Because on rereview of the original articles we could not find evidence of neurologic involvement, we omitted 2 of the 23 children listed by Weinberg et al 6 in their 1983 review of pediatric neurosarcoid. In the paper by Beier and Lahey 7 we could not find the child with a 7th-nerve palsy referenced by Weinberg et al In addition, in the 1956 series collected by McGovern and Merritt 8 we were unable to identify the child tabulated by Weinberg et al as having "facial dysphagia.…”

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

“…Involvement of the central nervous system (CNS) in sarcoidosis is rare, occurring in only 4% of children [15]. Seizures occur in 38% of children with CNS sarcoidosis while 21% have cranial nerve involvement, most often facial nerve palsy [16].…”