Phenotypes for disease #01749 (HDLS (leukoencephalopathy, diffuse hereditary, with spheroid (HDLS)), OMIM:221820)

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0000283592 Briefly, at age 46 this previously healthy manual worker suffered a progressive personality change (HP:0000751) with striking passivity and loss of responsibility at work and at home. At his first examination half a year after onset we observed a debilitating frontal syndrome with total loss of insight, along with discrete pyramidal (HP:0007256) and deep sensory signs. During the subsequent 2 years he was in a permanent hyperactive state (HP:0000752), incessantly walking, opening cupboards or clapping his hands, still with only moderate motor, sensory and extrapyramidal signs (HP:0002071). In the fourth year of his disease he had developed complete hemianopia (HP:0012377), and showed gradually increasing rigidity (HP:0002063) in all extremities, as well as primitive brain stem and grasp reflexes (HP:0002476). For 6 years he remained in a vegetative state (HP:0031358) with a general decortical type of rigidity, a weak doll’s eye reaction and spontaneous respiration of Cheyne-Stokes type (HP:0012196) until he succumbed from respiratory infections at 57 years of age. Five consecutive MRI examinations up to 26 months disease duration with DTI showed a symmetric leukoencephalopathy (HP:0002352) with an unusual feature, a progressive rim of decreased diffusion expanding centrifugally through the white matter from the periventricular area of the frontal and occipital horns, leaving apparently disorganized tissue behind the rim. - HDLS Familial, autosomal dominant 46y-57y - 46y - - Rita Guerreiro 00390053
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