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with vitamin E de ciency: (1) de cient fat absorption (eg cystic brosis, chronic cholestasis, short-bowel syndrome, and intestinal lymphangiectasia), (2) de cient fat transport (abetalipoproteinemia, hypobetalipoproteinemia, normotriglyceridemic abetalipoproteinemia, and chylomicron retention disease), and (3) a genetically based abnormality of vitamin E metabolism Patients with vitamin E de ciency usually present with progressive dif culty ambulating and impaired coordination of the hands59 62 Some individuals complain of weakness and sensory loss Dysarthria can also occur Physical examination is remarkable for ataxia of the trunk and upper and lower extremities59 62 There is prominent loss of proprioception and vibratory perception Muscle stretch re exes are reduced or absent Manual muscle testing can be dif cult secondary to the ataxia, but there can be proximal muscle weakness, suggesting a superimposed myopathic process Ocular examination may reveal ophthalmoplegia and retinopathy
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Vitamin E (alpha-tocopherol) levels in the serum are low With hyperlipidemia, the vitamin E level may be normal even when de ciency is present; therefore, the ratio of total serum vitamin E to the total serum lipid concentration is a more sensitive indicator of vitamin E de ciency in such cases63 NCS reveal reduced amplitudes or absent SNAPs57 62,64 The sensory nerve conduction velocities are normal or only slightly reduced Somatosensoryevoked potentials demonstrate normal peripheral nerve potentials with marked slowing and attenuation of central responses documenting slowing of central conduction with loss of posterior column bers65 Motor NCS are normal
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the small intestine Vitamin E is incorporated into chylomicrons and is transported to the liver In the liver, Vitamin E is transferred to very-low-density lipoproteins in a step requiring alpha-tocopherol transfer protein in 10 Vitamin E appears to have important roles in scavenging free radicals and in maintaining cell membrane structure71 Since vitamin E de ciency is usually due to factors other than insuf cient intake72 Vitamin E de ciency usually occurs in secondary lipid malabsorption or in uncommon disorders of vitamin E absorption or transport One hereditary disorder associated with Vitamin E de ciency is abetalipoproteinemia This is a rare autosomaldominant disorder characterized by steatorrhea, pigmentary retinopathy, acanthocytosis, and progressive ataxia73 In addition, individuals with cystic brosis may also have vitamin E de ciency secondary to steatorrhea There are also genetic forms of isolated vitamin E de ciency not associated with lipid malabsorption74,75 Mutation in the alpha-tocopherol transfer protein gene on chromosome 8q13 affects vitamin E absorption, impairing its incorporation in very-low-density lipoproteins (see 10)70,76 Vitamin E de ciency may also occur as a consequence of various cholestatic and hepatobiliary disorders as well as short bowel syndromes, resulting in the surgical treatment of intestinal disorders64,77,78
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The primary goal is to prevent progression, but improvement in neurological function may occur The speci c dose of vitamin E varies with the cause of de ciency72 With isolated vitamin E de ciency, patients are treated with 1500 6000 international units (IU) per day in divided doses In cases of chronic cholestasis, treatment begins with 50 IU/kg/d and the dose is increased in 50 IU/kg increments up to a 200 IU/kg/d to achieve normalization of the serum tocopherol to lipid ratio Patients with cystic brosis who are receiving oral pancreatic enzyme therapy require doses of 5 10 IU/kg/d Those with short bowel syndrome are given 300 5400 IU/d Abetalipoproteinemia is treated with vitamin E 150 300 IU/kg/d and vitamin A 15,000 20,000 IU/d
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Autopsy studies demonstrate swelling and degeneration of axons in the posterior columns and spinocerebellar tracts along with neuronal loss and lipofuscin accumulation in the gracile and cuneate nuclei60,66,67 The basal ganglia may be involved as well Sural nerve biopsies show nonspeci c changes and loss of large myelinated bers, axonal degeneration and regenerating clusters, occasional vacuoles in the myelin sheath, and breakup of the Schmidt Lanterman incisures, but little in the way of primary demyelination68,69
Polyneuropathy may occur following gastric/bariatric surgery for ulcer, cancer, or morbid obesity This usually occurs in the context of rapid, signi cant weight loss and recurrent, protracted vomiting14,79 82 The clinical picture is variable and may include acute or subacute sensory loss, burning feet, and generalized weakness that can resemble Guillian Barre syndrome, mononeuropathies,
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