Necrotizing sarcoid granulomatosis with neurologic lesions in a child

Beach, Richard; Corrin, B.; Scopes, J W; Graham, Elizabeth

doi:10.1016/s0022-3476(80)80431-8

Cited by 38 publications

(18 citation statements)

References 8 publications

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“…Necrotizing sarcoidosis, like typical sarcoidosis, is a diagnosis of exclusion and should not be made until all special studies including cultures have been performed to rule out an infectious cause. Although patients with confirmed extrapulmonary necrotizing sarcoidosis and a single case with presumed CNS involvement have been described in the literature (22), this report is the first to document histologically proven intracranial involvement. It also provides a rare example of the combined presentation of a case with typical sarcoidosis and subsequent necrotizing granulomatosis.…”

Section: Discussionmentioning

confidence: 78%

“…The vast majority involve the lungs (10 -15), with some cases involving skin and subcutaneous tissues (16), kidney (17), lacrimal gland and gastrointestinal tract (18), orbit (19), liver (20), and spinal column (21). A single report (22) described neurologic involvement and CNS lesions by imaging studies in a patient with biopsy-proven pulmonary and mediastinal disease. Thus, to our knowledge, we report with histologic confirmation the first case of NSG involving the intracranial compartment reported in the English literature.…”

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confidence: 99%

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Necrotizing Sarcoid Granulomatosis Mimicking an Intracranial Neoplasm: Clinicopathologic Features and Review of the Literature

2000

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We present a unique case of biopsy-proven necrotizing sarcoidosis involving the central nervous system (CNS) in a 52-year-old woman. The patient presented with a 3-month history of left-sided headache and sharp, shooting pains on the left side of her face. She also has a previous history of sarcoidosis, histopathologically confirmed on parotid gland biopsy 24 years before. Imaging studies of the present lesion revealed a 1.8 ؋ 1.4-cm mass in the left temporal lobe with signal intensity suggestive of meningioma or low-grade glial neoplasm. Surgical resection was initiated, and intraoperative consultation with frozen sections revealed granulomata. The lesion was biopsied, and surgical intervention was terminated. Permanent sections failed to reveal bacteria, mycobacteria, fungi, or foreign bodies. A diagnosis of necrotizing neurosarcoidosis was rendered. The patient was administered steroid therapy and clinically responded favorably. At the most recent follow-up almost 2 years later, there was no evidence of recurrence or progression. Necrotizing sarcoidosis has been reported most commonly in the lungs and rarely in other organ systems. We report the first histologically proven case involving the CNS as well as a rare example of sarcoidosis and necrotizing sarcoid granulomatosis in the same patient. Sarcoidosis and its necrotizing variant should be considered in the differential diagnosis of a granulomatous mass lesion involving the CNS, particularly in the context of a history of systemic disease.

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Section: Discussionmentioning

confidence: 78%

mentioning

confidence: 99%

Necrotizing Sarcoid Granulomatosis Mimicking an Intracranial Neoplasm: Clinicopathologic Features and Review of the Literature

2000

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“…NSG in childhood, to the current authors' knowledge, has been described in literature only four times and in only two of these cases [5,6] was the pulmonary involvement isolated, as in the case described here. In the other two cases [7,8] neurogical and ophthalmological lesions were encountered as well.…”

Section: Discussionmentioning

confidence: 92%

“…Experience in terms of therapy is unfortunately still limited. Data from adults suggest that corticosteroids and immunosuppressive therapy should be restricted to patients with progressive or severe NSG, particularly in cases with central nervous system involvement [4,7,9,22,23].…”

Section: Discussionmentioning

confidence: 99%

A 13-year-old female with shortness of breath and pleuritic chest pain

Aliberti

Falleni

Tarsia

et al. 2006

European Respiratory Journal

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A 13-yr-old Caucasian female was referred to the Dept of Paediatric Diseases (Havana, Cuba) with shortness of breath and pleuritic chest pain located in her right hemithorax. The pain had occurred the day before while doing physical activity and lasted ,1 h. The patient reported a 2-month history of recurrent episodes of pleuritic chest pain, which were either self-limited or relieved by paracetamol, as well as growing fatigue in the previous few weeks. Her past history was remarkable for a diagnosis of pneumonia after exposure to fumigation during a dengue haemorrhagic fever epidemic 2 yrs earlier, which was treated with steroids and antibiotics.On admission, the patient had no weight loss, no fever or dyspnoea. Physical examination showed decreased breathing sounds in the inferior part of both hemithoraces. Cardiovascular, abdominal and neurological examination, as well as ear, nose and ophthalmologic investigations were unremarkable. The chest radiograph showed bilateral pneumonia with moderate right-sided pleural effusion. The leukocyte count was 9.8610 9 cells?L -1 with 54% neutrophils and 32% lymphocytes. The erythrocyte sedimentation rate was 85 mm?h -1 . Serum chemistry, renal and liver function tests were in the normal range. Blood cultures were negative. Antibiotic therapy with ceftriaxone and amikacin was started. During the treatment the patient reported 2 days of moderate fever and various episodes of left-sided pleuritic chest pain, which occurred especially during forceful diaphragm contractions (i.e. sneezing, laughing and coughing).Following a short stay in the Cuban hospital, the patient was transferred to an Italian hospital (Como). Amikacin was discontinued and clarithromycin added. A new chest radiograph ( fig. 1), 14 days after the previous one, and a computed tomography (CT) scan of the thorax were performed. Echocardiography and abdominal ultrasound were both negative. An increased value of the tumour marker CA-125 (220 U?mL -1 , versus a normal value ,35 U?mL -1 ) was found. Microbiological and blood testing workup results are shown in table 1.After 1 week, the patient was admitted to a hospital in Milan where a thoracic CT scan ( fig. 2) and whole-body positron emission tomography (PET; fig. 3) were performed. The latter was obtained in order to identify any possible extrapulmonary focus of the disease. Further microbiological and blood testing is shown in table 1.Pulmonary function tests showed a restrictive ventilatory pattern with a total lung capacity 76% of predicted, a vital capacity of 2.66 L (75% pred), a forced expiratory volume in one second of 2.43 L (82% pred), a transfer factor of the lung for carbon monoxide of 6.52 mmol?min -1 ?kPa -1 (71% pred) and a transfer coefficient of the lung for carbon monoxide of 1.83 mmol?min -1 ?kPa -1 ?L -1 (94% pred).Fibreoptic flexible bronchoscopy did not reveal any endobronchial lesion. Cultures of bronchoalveolar lavage (BAL) for bacteria, including Mycobacterium tuberculosis, and for fungi, and a search for malignant cells were negative...

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“…El principal tratamiento para la neurosarcoidosis son los esteroides. La radioterapia y el tratamiento inmunosupresor, particularmente el metotrexato, la azatioprina, y la ciclosporina se han usado en casos resistentes (25,31). En nuestro caso la asociación de metotrexato permitió un buen control de la enfermedad y el disminuir rápidamente la dosis de glucocoticosteroides.…”

Section: Discussionunclassified

Sarcoidosis sistémica necrotizante con afectación pulmonar y del sistema nervioso central

Fernández

Callejas-Rubio

Fernández

et al. 2008

An. Med. Interna (Madrid)

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INTRODUCCIÓNLa sarcoidosis es una enfermedad multisistémica caracterizada por un proceso granulomatoso inflamatorio que afecta el pulmón (1) y otros órganos (2). Normalmente el diagnósti-co microscópico de sarcoidosis se hace cuando el anatomopatólogo reconoce granulomas epitelioides no necrotizantes en la biopsia. No obstante, en algunos casos puede haber necrosis (3) y la sarcoidosis puede ser confundida con un proceso infeccioso. Describimos un caso de un paciente varón que desarrolló sarcoidosis con afectación pulmonar y del sistema nervioso central cuyo estudio histológico demostró granulomas con necrosis lo que contribuyó a dificultar el diagnóstico. CASO APORTADOEl paciente era un varón de 31 años, trabajador del campo, fumador, con antecedentes de apendicectomía y absceso perianal que fue tratado quirúrgicamente. En el momento de la presentación, en enero de 2003, comenzó con mareos y pérdida de memoria. Los mareos consistían en vértigo rotacional episódicos con sensación de inestabilidad en la marcha, acompañados de sudoración, nauseas y vómitos. Tres meses más tarde tuvo una caída al suelo, con pérdida de conciencia, y movimientos tónico-clónicos, salivación excesiva, sin mordedura de lengua, con incontinencia urinaria y fecal, y con una obnubilación postcrisis de 48 horas. Se realizó en ese momento un electroencefalograma y un TAC craneal que fueron normales; fue diagnosticado de epilepsia y tratado con carbamazepina. Desde entonces, comenzó con debilidad en la pierna derecha, alteración en la marcha, y un importante empeoramiento del equilibrio, con frecuente caídas. También presentaba un habla no fluente, interrumpida y con déficits, confusión, pérdida de memoria episódica, dificultad para la comprensión de una conversación, empeoramiento de la escritura, problemas higiénicos e incontinencia de esfínteres. Su hermano, con quien él convivía, había sido diagnosticado recientemente de sarcoidosis con afectación pulmonar y de la piel. La exploración física era normal, sin fiebre ni signos de afectación pulmonar. La exploración neurológica mostraba un paciente alerta, orientado en espacio y tiempo, aunque bradipsíquico. La exploración de los pares craneales era normal, salvo nistagmo horizontal hacia el lado derecho. Las pupilas eran simétricas y reactivas. El fondo de ojo era normal. No había rigidez de nuca. Tenía una paresia grado 4+/5 en el brazo izquierdo y en ambas piernas. El tono era normal. Los reflejos estaban exagerados en ambas piernas. El reflejo cutáneoplantar era [0212-7199 (2008) RESUMENLa sarcoidosis es una enfermedad sistémica que se diagnostica con la presencia de granulomas no necrotizantes en una biopsia. Sin embargo, hay variantes como la sarcoidosis con granulomas necrotizantes o la sarcoidosis nodular en las que pueden aparecer zonas de necrosis que hacen que el diagnóstico diferencial con procesos infecciosos sea especialmente difícil.Describimos un caso de un paciente, con afectación pulmonar y del SNC, en quien la presencia de granulomas necrotizantes hizo orientar el diagnó...

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Necrotizing sarcoid granulomatosis with neurologic lesions in a child

Cited by 38 publications

References 8 publications

Necrotizing Sarcoid Granulomatosis Mimicking an Intracranial Neoplasm: Clinicopathologic Features and Review of the Literature

Necrotizing Sarcoid Granulomatosis Mimicking an Intracranial Neoplasm: Clinicopathologic Features and Review of the Literature

A 13-year-old female with shortness of breath and pleuritic chest pain

Sarcoidosis sistémica necrotizante con afectación pulmonar y del sistema nervioso central

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