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Neurosarcoidosis

Neurosarcoidosis. Betsy D. Hughes, BS, J. Ned Pruitt II, MD, and John R. Vender, MD Contemporary Neurosurgery Vol. 29, Num. 3, Feb. 15, 2007 R 石孟申 /VS 高定憲. Sarcoidosis. Inflammatory multisystem disorder Noncaseating granulomas Lung: most common

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Neurosarcoidosis

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  1. Neurosarcoidosis Betsy D. Hughes, BS, J. Ned Pruitt II, MD, and John R. Vender, MD Contemporary Neurosurgery Vol. 29, Num. 3, Feb. 15, 2007 R石孟申/VS高定憲

  2. Sarcoidosis • Inflammatory multisystem disorder • Noncaseating granulomas • Lung: most common • Skin, eyes, liver, lymph nodes • CNS: less common but severe • Isolated CNS lesions: mimic brain and spinal neoplasm.

  3. Prevalence • Age: 20 to 40 y/o • 10 to 40 cases per 100,000 population in the United States and Europe • African-Americans : Caucasians • 10:1 to 17:1 • Nervous system involvement • 5% to 15%: patients with sarcoidosis • 25%: Postmortem studies

  4. Pathophysiology • Non caseaing granuloma: epithelioid macrophages, lymphocytes, monocytes, fibroblasts • Exaggerated cellular immune response • Activation of the Th1 T lymphocyte cell line. • By IL-4 and CD28 • Release INF-r: macrophages

  5. Clinical Manifestations • Cranial Neuropathies • Hydrocephalus • Meningeal Disease • Intracranial Parenchymal Disease • Spinal Cord Lesions • Peripheral Neuropathies • Skeletal Muscle Involvement

  6. Cranial Neuropathies • Most common presentation of neurosarcoidosis • Heerfordt syndrome: Acute neurosarcoidosis • Facial nerve palsy • Parotid gland enlargement • Anterior uveitis • Fever

  7. Hydrocephalus • Meningeal involvement • Infiltration of the ependyma or choroid plexus • Intrinsic obstruction of the ventricular system • Communicating or noncommunicating

  8. Meningeal Disease • Present like aseptic meningitis or a mass lesion • Pleocytosis: 70% of patients • Elevated Protein: 80% • Reduced glucose: 10% • Thickened, chronic meningitis

  9. Intracranial Parenchymal Disease • Sarcoid encephalopathy • seizures • mood disturbances • cognitive impairment • psychosis • personality changes

  10. Intracranial Parenchymal Disease • Hypothalamus, infundibulum, and posterior lobe of the pituitary • direct tissue involvement • changes related to adjacent basal meningeal disease • Enhanced MRI can show the enlargement of these structures; • mimic a pituitary tumor or a glioma

  11. Spinal Cord Lesions • Cervical and thoracic intramedullary lesions are the most common. • mimic spinal cord neoplasms both clinically and radiologically. • Imaging studies • Enhancement of the lesions • enlargement of the cord • surrounding edema.

  12. Peripheral Neuropathies • Paresthesias and radiculopathy • Sensorimotor, Motor, Sensory • Small or Large • Autonomic • NCV

  13. Skeletal Muscle Involvement • Extremely common and usually asymptomatic • Weakness, pain, and tenderness • Firm, palpable nodules and cramps, with atrophy and contractures • EMG + Muscle biopsy

  14. Diagnosis • Zajicek et al. proposed diagnostic criteria for three categories of neurosarcoidosis • Certain neurosarcoidosis • Probable neurosarcoidosis • Possible neurosarcoidosis

  15. Certain Neurosarcoidosis • Biopsy of nervous tissue • noncaseating granulomas • Other possible diagnoses are excluded.

  16. Probable Neurosarcoidosis • Clinical presentation • Laboratory evidence demonstrates signs of CNS inflammation • Systemic sarcoidosis. • Other diagnoses are excluded

  17. Possible Neurosarcoidosis • Clinical presentation • Other diagnoses are excluded

  18. Diagnosis • CXR, Chest CT, gallium-67 scan, bronchoalveolar lavage • PFT, Ophthalmologic • Serum ACE (Angiotensin-converting enzyme) • MRI of the muscles, PET, 24-hr urine calcium, VEP, NCV, EMG

  19. CNS inflammation • Elevation of ACE • Normal to low glucose • Elevated immunoglobulin G • Oligoclonal bands • Elevation of lysozyme and β2-microglobulin

  20. Brain MRI • Most sensitive imaging study for neurosarcoidosis. • Irregular leptomeningeal thickening and enhancement • the base of the brain, pituitary and infundibulum. • Periventricular abnormalities, white matter lesions, hydrocephalus, and cranial nerve enhancement.

  21. Treatment • Goal of therapy: • Decrease fibrosis and ischemia of the tissue • First-line: corticosteroids • Methotrexate, • infliximab

  22. Corticosteroids • Facial palsy: 1 week of prednisone followed by a 1-week taper. • Parenchymal disease: high-dose prednisone for 2 to 4 weeks, followed by a taper. • Severe acute neurological symptoms : IV methylprednisolone for 3 days, followed by 2 to 4 weeks of prednisone with taper.

  23. Surgery • Indications: • Hydrocephalus • Life-threatening mass lesions causing IICP • VP shunt: • Stereotactic guidance • Multiple shunts and revision procedures • Endoscopic fenestration of intraventricular adhesions and septae • Increased infection rate

  24. Conclusion • Biopsy: after extensive medical and laboratory testing • Corticosteroids usually provides a favorable outcome. • Aggressive surgical intervention rarely is indicated • V-P shunt: appropriate with hydrocephalus secondary to neurosarcoidosis.

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