Sarcoidosis of the central nervous system

Abstract

SUMMARY Six patients with sarcoidosis of the central nervous system are described. Pathological confirmation was obtained by brain biopsy in two patients and at necropsy in two; in two patients the diagnosis was presumptive and was made on the evidence of multisystem involvement. The symptomatology, methods of diagnosis, and results of treatment are discussed.

Neurosarcoid Presents Differently in Children Than in Adults

“…Twenty-four percent (7 of 29) had mass lesions or focal edema on imaging: One 8-year-old child with seizures had a "ring cortical lesion" on computed tomography (CT) 9 ; our case (age 9) had papilledema and swollen frontal gyri on MRI; one 12-year-old child had nodular lesions in the optic chiasm and right sylvian fissure on MRI 10 ; a 13-year-old child had a pontine mass on MRI 11 ; a 16-year-old child had hemiparesis and a left frontal mass 12 ; a 17-year-old child with seizures had a temporal mass on MRI 13 ;…”

Neurosarcoid Presents Differently in Children Than in Adults

“…Twenty-four percent (7 of 29) had mass lesions or focal edema on imaging: One 8-year-old child with seizures had a "ring cortical lesion" on computed tomography (CT) 9 ; our case (age 9) had papilledema and swollen frontal gyri on MRI; one 12-year-old child had nodular lesions in the optic chiasm and right sylvian fissure on MRI 10 ; a 13-year-old child had a pontine mass on MRI 11 ; a 16-year-old child had hemiparesis and a left frontal mass 12 ; a 17-year-old child with seizures had a temporal mass on MRI 13 ;…”

Sarcoidosis presenting as hiccups

“…Four were successfully treated with corticosteroids and one patient had a poor response to treatment, resulting in death. Among the five cases with hiccups thought to be due to CNS involvement, all [1,3,5,7,8] showed neurological abnormalities, elevated protein and/or cell count within the cerebrospinal fluid, and all had abnormal brain imaging. Kondo et al's case, reporting hiccups caused by CNS sarcoid, is perplexing as there were no neurological symptoms reported and the CT scan and magnetic resonance imaging (MRI) of the brain were normal [2].…”

Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

“…The prevalence of clinically manifest sarcoidosis of the nervous system in patients with the systemic disease has ranged from 1% (Douglas and Maloney, 1973), to 4.3% (Slade, 1979), to 16% (Maycock et al, 1963. In postmortem series, neurosarcoidosis has been reported in approximately 15% (Aszkanazy, 1952;Ricker and Clark, 1949;Waxman and Sher, 1979).…”

Neurosarcoid Presents Differently in Children Than in Adults

“…Twenty-four percent (7 of 29) had mass lesions or focal edema on imaging: One 8-year-old child with seizures had a "ring cortical lesion" on computed tomography (CT) 9 ; our case (age 9) had papilledema and swollen frontal gyri on MRI; one 12-year-old child had nodular lesions in the optic chiasm and right sylvian fissure on MRI 10 ; a 13-year-old child had a pontine mass on MRI 11 ; a 16-year-old child had hemiparesis and a left frontal mass 12 ; a 17-year-old child with seizures had a temporal mass on MRI 13 ;…”

Sarcoidosis presenting as hiccups

“…Four were successfully treated with corticosteroids and one patient had a poor response to treatment, resulting in death. Among the five cases with hiccups thought to be due to CNS involvement, all [1,3,5,7,8] showed neurological abnormalities, elevated protein and/or cell count within the cerebrospinal fluid, and all had abnormal brain imaging. Kondo et al's case, reporting hiccups caused by CNS sarcoid, is perplexing as there were no neurological symptoms reported and the CT scan and magnetic resonance imaging (MRI) of the brain were normal [2].…”

Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

“…The prevalence of clinically manifest sarcoidosis of the nervous system in patients with the systemic disease has ranged from 1% (Douglas and Maloney, 1973), to 4.3% (Slade, 1979), to 16% (Maycock et al, 1963. In postmortem series, neurosarcoidosis has been reported in approximately 15% (Aszkanazy, 1952;Ricker and Clark, 1949;Waxman and Sher, 1979).…”

Neurosarcoid Presents Differently in Children Than in Adults

“…Twenty-four percent (7 of 29) had mass lesions or focal edema on imaging: One 8-year-old child with seizures had a "ring cortical lesion" on computed tomography (CT) 9 ; our case (age 9) had papilledema and swollen frontal gyri on MRI; one 12-year-old child had nodular lesions in the optic chiasm and right sylvian fissure on MRI 10 ; a 13-year-old child had a pontine mass on MRI 11 ; a 16-year-old child had hemiparesis and a left frontal mass 12 ; a 17-year-old child with seizures had a temporal mass on MRI 13 ;…”

Sarcoidosis presenting as hiccups

“…Four were successfully treated with corticosteroids and one patient had a poor response to treatment, resulting in death. Among the five cases with hiccups thought to be due to CNS involvement, all [1,3,5,7,8] showed neurological abnormalities, elevated protein and/or cell count within the cerebrospinal fluid, and all had abnormal brain imaging. Kondo et al's case, reporting hiccups caused by CNS sarcoid, is perplexing as there were no neurological symptoms reported and the CT scan and magnetic resonance imaging (MRI) of the brain were normal [2].…”

Pathobiology of Neurosarcoidosis and Clinicopathologic Correlation

“…The prevalence of clinically manifest sarcoidosis of the nervous system in patients with the systemic disease has ranged from 1% (Douglas and Maloney, 1973), to 4.3% (Slade, 1979), to 16% (Maycock et al, 1963. In postmortem series, neurosarcoidosis has been reported in approximately 15% (Aszkanazy, 1952;Ricker and Clark, 1949;Waxman and Sher, 1979).…”