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MAJOR CATEGORIES OF NEUROLOGIC DISEASE
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nated oil to hypothyroid women before and during the rst trimester of pregnancy prevents sporadic and endemic cretinism, as already noted Treatment begun during the second trimester protects the fetal brain to a varying degree Treatment that is started after the beginning of the third trimester does not improve the neurologic status, although head growth and statural development may improve slightly (Cao et al) In sporadic cretinism, if the condition is recognized early, say at birth, and treated consistently with thyroid hormones, statural and mental development can be stimulated to normal or near-normal levels The extent of recovery depends on the severity and duration of intrauterine hypothyroidism, ie, its duration before treatment was begun and the adequacy of therapy In most patients, some degree of mental de ciency persists throughout life Pancreatic Encephalopathy This term was introduced by Rothermich and von Haam in 1941 to describe what they considered to be a fairly uniform clinical state in patients with acute abdominal symptoms referable to pancreatic disease, mainly pancreatitis The encephalopathy, as they described it, consisted of an agitated, confused state, sometimes with hallucinations and clouding of consciousness, dysarthria, and changing rigidity of the
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limbs all of which uctuated over a period of hours or days Coma and quadriplegia have been reported At autopsy, a variety of lesions have been described; two cases have had central pontine myelinolysis and others have had small foci of necrosis and edema, petechial hemorrhages, and demyelination scattered through the cerebrum, brainstem, and cerebellum These have been uncritically attributed to the action of released lipases and proteases from the action of pancreatic enzymes (see review of this subject by Sharf and Levy) The term pancreatic encephalopathy has also been applied to a depressive illness that seems to occur with disproportionate frequency before the symptoms of a pancreatic cancer become apparent Also notable in our experience are numerous cases of pancreatic cancer and sequential cerebral emboli from nonbacterial thrombotic (marantic) endocarditis The status of pancreatic encephalopathy, in the authors opinion, is uncertain Pallis and Lewis also express reservations and suggest that before such a diagnosis can be seriously entertained in a patient with acute pancreatitis, one must exclude delirium tremens, shock, renal failure, hypoglycemia, diabetic acidosis, hyperosmolality, and hypocalcemia or hypercalcemia any one of which may complicate the underlying disease Other cases conform to the encephalopathy of multiorgan failure, discussed earlier
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ADAMS RD, FOLEY JM: The neurological disorder associated with liver disease Res Publ Assoc Res Nerv Ment Dis 32:198, 1953 ADAMS RD, VICTOR M, MANCALL EL: Central pontine myelinolysis Arch Neurol Psychiatry 81:154, 1959 AIKAWA N, SHINOZAWA Y, ISHIBIKI K, et al: Clinical analysis of multiple organ failure in burned patients Burns 13:103, 1987 ALFREY AC, LEGENDRE GR, KAEHNY WD: The dialysis encephalopathy syndrome: Possible aluminum intoxication N Engl J Med 294:184, 1976 AMES A, WRIGHT RL, KOWADA M, et al: Cerebral ischemia: II The nore ow phenomenon Am J Pathol 52:437, 1968 ARIEFF AI: Hyponatremia associated with permanent brain damage Adv Intern Med 21:325, 1987 ARIEFF AI: Hyponatremia, convulsions, respiratory arrest, and permanent brain damage after elective surgery in healthy women N Engl J Med 314:1529, 1986 AUER RN: Progress review: Hypoglycemic brain damage Stroke 17:699, 1986 AUSTEN FK, CARMICHAEL MW, ADAMS RD: Neurologic manifestations of chronic pulmonary insuf ciency N Engl J Med 257:579, 1957 BASILE AS, HUGHES RD, HARRISON PM, et al: Elevated brain concentrations of 1,4-benzodiazepines in fulminant hepatic failure N Engl J Med 325:473, 1991 BERNARD Sa, GRAY TW, BUIST MD, et al: Treatment of comatose survivors of out-of-hospital cardiac arrest with induced hypothermia N Engl J Med 346:557, 2002 BHATIA MP, BROWN P, GREGORY R, et al: Progressive myoclonic ataxia associated with coeliac disease Brain 118:1087, 1995 BOLTON CF, YOUNG GB: Neurological Complications of Renal Disease Boston, Butterworth, 1990 BOLTON C, YOUNG GB, ZOCHODNE DW: The neurological complications of sepsis Ann Neurol 33:94, 1993 BRAIN L, JELLINEK EH, BALL K: Hashimoto s disease and encephalopathy Lancet 2:512, 1966 BURCAR PJ, NORENBERG MD, YARNELL PR: Hyponatremia and central pontine myelinosis Neurology 27:223, 1977 BURN DJ, BATES D: Neurology and the kidney J Neurol Neurosurg Psychiatry 65:810, 1998 BUTTERWORTH RF, GIGUIERE JF, MICHAUD J, et al: Ammonia: Key factor in the pathogenesis of hepatic encephalopathy Neurochem Pathol 6:1, 1987 CAO X-Y, JIAN GX-M, DOU Z-H, et al: Timing of vulnerability of the brain to iodine de ciency in endemic cretinism N Engl J Med 331:1739, 1994 CAVANAGH JB: Liver bypass and the glia, in Plum F (ed): Brain dysfunction in metabolic disorders Res Publ Assoc Res Nerv Ment Dis 53:13, 1974 CHOI DW, ROTHMAN SM: The role of glutamate neurotoxicity in hypoxicischemic neuronal death Annu Rev Neurosci 13:171, 1990 CHOI IS: Delayed neurologic sequelae in carbon monoxide intoxication Arch Neurol 40:433, 1983 CHONG JY, ROWLAND LP, UTIGER RD: Hashimoto encephalopathy: Syndrome or myth Arch Neurol 60:164, 2003 COOKE WT, SMITH WT: Neurologic disorders associated with adult coeliac disease Brain 89:683, 1966 CREMER GM, GOLDSTINE NP, PARIS J: Myxedema and ataxia Neurology 19:37, 1969 CROSS AH, GOLUMBEK PT: Neurologic manifestations of celiac disease Neurology 60:1566, 2003 DAHLQUIST NR, PERRAULT J, CALLAWAY CW: D-Lactic acidosis and encephalopathy after jejunoileostomy: Response to overfeeding and to fasting in humans Mayo Clin Proc 59:141, 1984 DELONG GR, STANBURY JB, FIERRO-BENITEZ R: Neurological signs in congenital iodine-de ciency disorder (endemic cretinism) Dev Med Child Neurol 27:317, 1985 DIMAURO S, TONIN P, SERVIDEI S: Metabolic myopathies, in Rowland LP, DiMauro S (eds): Handbook of Clinical Neurology Vol 18 New York, Elsevier, 1992, pp 479 526 DOOLING EC, RICHARDSON EP JR: Delayed encephalopathy after strangling Arch Neurol 33:196, 1976 EAYRS JT: In uence of the thyroid on the central nervous system Br Med Bull 16:122, 1960 FARRELL RJ, KELLY CP: Celiac sprue N Engl J Med 346:180, 2002 FERRACCI F, MORETT OG, CANDEAGO RM, et al: Antithyroid antibodies in the CSF Their role in the pathogenesis of Hashimoto s encephalopathy Neurology 60:712, 2003
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