Brain Diseases, Metabolic
| Definition: |
Acquired or inborn metabolic diseases that produce brain dysfunction or damage. These include primary (i.e., disorders intrinsic to the brain) and secondary (i.e., extracranial) metabolic conditions that adversely affect cerebral function. |
| Notes: |
GEN; avoid: prefer specifics; DF: BRAIN DIS METAB |
| Also Called: |
Brain Diseases, Metabolic, Acquired,Central Nervous System Metabolic Disorders,Metabolic Disorder, Central Nervous System, Acquired,Acquired Metabolic Diseases, Nervous System |
| Previously Indexed: |
Brain Diseases (1966-1976) |
Brain Diseases, Metabolic Categories.
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Brain Diseases, Metabolic, Inborn - Errors of brain metabolism resulting from inborn, primarily enzymatic, deficiency states. The majority of these conditions are familial, however spontaneous mutation may also occur. Deficient enzyme activity may cause brain dysfunction by leading to the accumulation of substrate, reduced product formation, or by causing metabolism to occur through alternate pathways. |
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Kernicterus - A term used pathologically to describe BILIRUBIN staining of the BASAL GANGLIA; BRAIN STEM; and CEREBELLUM and clinically to describe a syndrome associated with HYPERBILIRUBINEMIA. Clinical features include athetosis, MUSCLE SPASTICITY or hypotonia, impaired vertical gaze, and DEAFNESS. Nonconjugated bilirubin enters the brain and acts as a neurotoxin, often in association with conditions that impair the BLOOD-BRAIN BARRIER (e.g., SEPSIS). This condition occurs primarily in neonates (INFANT, NEWBORN), but may rarely occur in adults. (Menkes, Textbook of Child Neurology, 5th ed, p613) |
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Myelinolysis, Central Pontine - A demyelinating condition affecting the PONS and characterized clinically by an acute progressive QUADRIPLEGIA; DYSARTHRIA; DYSPHAGIA; and alterations of consciousness. Pathologic features include prominent demyelination in the central PONS with sparing of axons and neurons. This condition is usually associated with systemic disorders such as HYPONATREMIA; chronic ALCOHOLISM; LIVER FAILURE; severe BURNS; malignant NEOPLASMS; hemorrhagic PANCREATITIS; HEMODIALYSIS; and SEPSIS. The rapid medical correction of hyponatremia has been cited as a cause of this condition. (From Adams et al., Principles of Neurology, 6th ed, pp1125-6) |
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Wernicke Encephalopathy - An acute neurological disorder characterized by the triad of ophthalmoplegia, ataxia, and disturbances of mental activity or consciousness. Eye movement abnormalities include nystagmus, external rectus palsies, and reduced conjugate gaze. THIAMINE DEFICIENCY and chronic ALCOHOLISM are associated conditions. Pathologic features include periventricular petechial hemorrhages and neuropil breakdown in the diencephalon and brainstem. Chronic thiamine deficiency may lead to KORSAKOFF'S SYNDROME. (Adams et al., Principles of Neurology, 6th ed, pp1139-42; Davis & Robertson, Textbook of Neuropathology, 2nd ed, pp452-3) |
Brain Diseases, Metabolic Definitions and Terms
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