Neurologic dysfunction

Fatal hereditary disorder that typically presents in the neonatal period. Clinical features include an array of hepatic, renal and neurological dysfunctions. Patients with Zellweger syndrome rarely survive the first year of life. The disease is caused by mutations in the Pex proteins leading to an defective import of peroxisomal matrix proteins and consequently to a loss of most peroxisomal metabolic pathways. 

Curto R> Voit EO, Cascante M Analysis of abnormalities in purine metabolism leading to gout and to neurological dysfunctions in man. Biochem J 1998 3 29 477. 

E Tocopherols, tocotrienols Antioxidant, especially in cell membranes Extremely rare—serious neurologic dysfunction... 

Youdim, K.A. and Joseph, J.A., A possible emerging role of phytochemicals in improving age-related neurological dysfunctions a multiplicity of effects, Eree Radio. Biol. Med., 30, 583, 2001. 

High doses may contribute to postresuscitation myocardial and neurological dysfunction... 

Patients usually have multiple signs of neurologic dysfunction, and the specific deficits are determined by the area of the brain involved. 

Although not typically life-threatening, spinal cord compression is a true oncologic emergency because delays in treatment by mere hours may lead to permanent neurologic dysfunction. It is therefore incumbent on practitioners to... 

Hereditary triose phosphate isomerase (TPI) deficiency is an autosomal recessive disorder that has the most severe clinical manifestations of the erythroenzy-mopathies, including hemolytic anemia, neurological dysfunction, sudden cardiac death, and increased susceptibility to infection. Since the first description by Schneider et al. (S10), more than 25 unrelated families have been reported (Fll). Cases of decreased TPI activities associated with cat cry syndrome and pancytopenia were reported, whereas the correlation between TPI deficiency and these disorders was not clear. Although the degree of anemia is variable, most patients require blood transfusions. Neurological involvement, such as paraparesis, weakness, and hypotonia, is progressive in most cases. No specific therapy is available for the neuropathic manifestations of the disease, and most severely affected children fail to survive beyond the age of 5 years. 

Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA, Scherzinger E, Wanker EE, Mangiarini L, Bates GP. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997 90 537-548. 

People with Neurologic Dysfunction or Kidney Disease. This population is unusually susceptible to lead exposure. The neurologic and renal systems are the primary target organs of lead intoxication, which may become overburdened at much lower threshold concentrations to elicit manifestations of lead intoxication (Benetou-Marantidou et al. 1988 Chisolm 1962, 1968 Lilis et al. 1968 Pollock and Ibels 1986). 

Patients usually have multiple signs of neurologic dysfunction on physical examination. The specific deficits observed depend upon the area of the brain involved. Hemi- or monoparesis and hemisensory deficits are common. Patients with posterior circulation involvement may present with vertigo and diplopia. Anterior circulation strokes commonly result in aphasia. Patients may also experience dysarthria, visual field defects, and altered levels of consciousness. 

The goals of treatment for acute stroke are to (1) reduce the ongoing neurologic injury and decrease mortality and long-term disability (2) prevent complications secondary to immobility and neurologic dysfunction and (3) prevent stroke recurrence. 

Increase in TXA2 synthesis at 5 min and 1 week of reperfusion suggests role in acute events and later stages of neurological dysfunction (Shohami et al., 1987). TXA2 synthetase inhibitor decreases vasospasm and reduces neurological deterioration after subarachnoid hemorrhage (Tokiyoshi et al., 1991). 

Neurotoxicity. No clinical signs of central nervous system toxicity or histological alterations of nervous system organs and tissues were observed in rats or mice in the NCI (1978) chronic oral bioassay. Tests for neurotoxicity in animals may be appropriate if there is clinical evidence of neurological dysfunction in general oral or dermal toxicity studies of I 2-diphenylhydrazine. 

The latency period between exposure and onset of symptoms ranges from hours to days. Initial symptoms are lethargy, somnolence, slurred speech, ataxia, dysmetria, and visual disturbances. Neurological dysfunction may progress to convulsions, coma, and death. If... 

In humans, the most important effect associated with inhalation exposure to 2-hexanone is neurological dysfunction, most commonly observed as peripheral neuropathy. Widespread attention was brought to this phenomenon after a 1973 outbreak of distal neuropathy in an Ohio fabric... 

Succinyl coenzyme A 3-oxo acid transferase catalyses the transformation of ACAC into acetoacetyl coenzyme A in the mitochondria of extra-hepatic tissues. This enzyme defect may be suggested in cases of severe ketoacidosis often associated with neurologic dysfunction [16]. 

However, these drugs are now more commonly— and more accurately—called neuroleptics or antipsy-chotics. As opposed to medications prescribed for sedation, the neuroleptics often produce signs of neurological dysfunction, such as extrapyrimidal effects (involuntary movements such as Parkinson-like tremors and other abnormal movements). The term antipsy-chotics is sometimes used because these drugs are generally used to treat symptoms of paranoia, psychosis, or serious distortions in the perception of reality, such as hallucinations or delusions. The neuroleptics are not typically drugs of abuse. 

MS is an autoimmune disease that attacks the myelin sheath of oligodendrocytes around the neuronal axons. This allows the axonal cytoskeleton to be damaged, bringing about secondary axonal loss and persisting neurological dysfunction. The characteristic pathology is of a lesion or plaque in the CNS white matter, formed by inflammation and demyelination and these can be classified into active, chronic active, or chronic silent plaques [86]. 

Kamel F, Hoppin JA (2004) Association of pesticide exposure with neurologic dysfunction and disease. Environ Health Perspect 112 950-958... 

Deficiencies of vitamin B12 can result from either low dietary levels or, more commonly, from poor absorption of the vitamin due to the failure of gastric parietal cells to produce intrinsic factor (as in pernicious anemia) or to a loss of activity of the receptor needed for intestinal uptake of the vitamin.5 Nonspecific malabsorption syndromes or gastric resection can also cause vitamin B12 deficiency. The vitamin may be administered orally (for dietary deficiencies), or intramuscularly or deep subcutaneously (for pernicious anemia). [Note Folic acid administration alone reverses the hematologic abnormality and thus masks the B12 deficiency, which can then proceed to severe neurologic dysfunction and disease. Therefore, megaloblastic anemia should not be treated with folic acid alone, but rather with a combination of folate and vitamin B12.] Therapy must be continued for the remainder of the life of a patient suffering from pernicious anemia. There are no known adverse effects of this vitamin. 

Thalamic stroke should be considered when there is a sudden onset of behavioral disturbance. The diagnosis is often missed since patients are thought to have primary psychiatric disorders, especially when neurological dysfunction is lacking. Distinct behavioral patterns can be delineated on the basis of the four main arterial thalamic territories (Schmahmann 2003 Carrera and Bogousslavsky 2006) ... 

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