Phenotypes for disease #01638 (ALXDRD (Alexander's disease), OMIM:203450)

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0000171383 - alexander disease - Familial, autosomal dominant - - - - - Gemeinschaftspraxis für Humangenetik Dresden 00226256
0000346283 see paper; ..., 5.4y-deceased; progressive megalencephaly, OFC SD+3; 4m-psychomotor degradation; recurrent seizures; MRI rostrocaudal gradient abnormal white matter signal; biopsy Rosenthal fibers; feeding difficulties; respiratory difficulties; neuropathology infantile disease Alexander disease ALXDRD Unknown 5.4y - 4m - - Johan den Dunnen 00457835
0000346284 see paper; ..., OFC SD+1; 8m-delayed psychomotor development; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Isolated (sporadic) 1.5y - 4m - - Johan den Dunnen 00457836
0000346285 see paper; ..., OFC SD+1; 1d-delayed psychomotor development; 7y-psychomotor degradation; occasional seizures; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Isolated (sporadic) 7.5y - 1d - - Johan den Dunnen 00457837
0000346286 see paper; ..., OFC SD+1.5; 8m-delayed psychomotor development; occasional seizures; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Unknown 2.6y - 8m - - Johan den Dunnen 00457838
0000346287 see paper; ..., OFC SD+0.5; 6m-delayed psychomotor development; occasional seizures; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Isolated (sporadic) 4.7y - 6m - - Johan den Dunnen 00457839
0000346288 see paper; ..., OFC SD+1.5; 6m-delayed psychomotor development; 8y-psychomotor degradation; occasional seizures; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Unknown 20y - 6m - - Johan den Dunnen 00457840
0000346289 see paper; ..., progressive megalencephaly, OFC SD+3; 6m-delayed psychomotor development; recurrent seizures; MRI rostrocaudal gradient abnormal white matter signal infantile disease Alexander disease ALXDRD Isolated (sporadic) 5.5y - 3m - - Johan den Dunnen 00457841
0000346290 see paper; ..., progressive megalencephaly, OFC SD+3; 1d-delayed psychomotor development; recurrent seizures; MRI rostrocaudal gradient abnormal white matter signal infantile disease Alexander disease ALXDRD Unknown 2.5y - 1d - - Johan den Dunnen 00457842
0000346291 see paper; ..., OFC SD+1; 1m-delayed psychomotor development; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Unknown 3.5y - 1m - - Johan den Dunnen 00457843
0000346292 see paper; ..., progressive megalencephaly, OFC SD+3; 6m-delayed psychomotor development; occasional seizures; MRI rostrocaudal gradient abnormal white matter signal infantile disease Alexander disease ALXDRD Isolated (sporadic) 2.2y - 6m - - Johan den Dunnen 00457844
0000346293 see paper; ..., OFC SD+3.5; 6m-delayed psychomotor development; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Isolated (sporadic) 2y - 6m - - Johan den Dunnen 00457845
0000346294 see paper; ..., 4.5y-deceased; OFC SD+1; 12m-delayed psychomotor development; 13m-psychomotor degradation; recurrent seizures; MRI abnormalities suggestive of Alexander disease; neuropathology infantile disease Alexander disease ALXDRD Isolated (sporadic) 4.5y - 9m - - Johan den Dunnen 00457846
0000346295 see paper; ..., OFC SD+1.5; 18m-delayed psychomotor development; 6y-psychomotor degradation; occasional seizures; MRI abnormalities suggestive of Alexander disease infantile disease Alexander disease ALXDRD Unknown 8y - 18m - - Johan den Dunnen 00457847
0000346296 see paper; ..., 1.2y-deceased; progressive megalencephaly, OFC SD+3; 3m-psychomotor degradation; occasional seizures; MRI rostrocaudal gradient abnormal white matter signal; Rosenthal fibers; feeding difficulties; respiratory difficulties; neuropathology infantile disease Alexander disease ALXDRD Isolated (sporadic) 1.2y - 3m - - Johan den Dunnen 00457848
0000346297 see paper; ..., 2.5y-deceased; progressive megalencephaly, OFC SD+4; 4m-delayed psychomotor development; 8m-psychomotor degradation; severe seizures (refractory); MRI rostrocaudal gradient abnormal white matter signal; Rosenthal fibers; feeding difficulties; respiratory difficulties; hydrocephalus; neuropathology infantile disease Alexander disease ALXDRD Unknown 2.5y - 4m - - Johan den Dunnen 00457849
0000346299 see paper; ..., 40w-birth, weight 2550g (−1.6SD), length 46cm (−2.0SD), OFC 31cm (−1.8SD); developmental milestones slightly delayed, 5m-head control, 12m-stand, 20m-walk, 24m-first speech; 5y-first seizure infantile-juvenile form Alexander disease ALXDRD Unknown 06y - - - - Johan den Dunnen 00457851
0000346300 see paper; ..., 14y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 14y - 3m - - Johan den Dunnen 00457852
0000346301 see paper; ..., 48y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 48y - 10y - - Johan den Dunnen 00457853
0000346302 see paper; ..., 6y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Isolated (sporadic) 6y - 13m - - Johan den Dunnen 00457854
0000346303 see paper; ..., 11y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 11y - 17m - - Johan den Dunnen 00457855
0000346304 see paper; ..., 10y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Isolated (sporadic) 10y - <1y - - Johan den Dunnen 00457856
0000346305 see paper; ..., 4y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Isolated (sporadic) 4y - <1y - - Johan den Dunnen 00457857
0000346306 see paper; ..., 11m-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 11m - 4m - - Johan den Dunnen 00457858
0000346307 see paper; ..., 6y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Isolated (sporadic) 6y - 2m - - Johan den Dunnen 00457859
0000346308 see paper; ..., 7y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 7y - 10m - - Johan den Dunnen 00457860
0000346309 see paper; ..., 8y-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Isolated (sporadic) 8y - 3m - - Johan den Dunnen 00457861
0000346310 see paper; ..., 4m-deceased, leukoencephalopathy, macrocephaly, seizures, psychomotor retardation Alexander disease ALXDRD Unknown 4m - 1d - - Johan den Dunnen 00457862
0000346313 no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; pathology from biopsy/autopsy; MRI brain atypical Alexander disease, adult ALXDRD Unknown 43y - 21y - - Johan den Dunnen 00457863
0000346314 macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 2y - 3m - - Johan den Dunnen 00457864
0000346315 no macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 9y - 7d - - Johan den Dunnen 00457865
0000346316 10y7m-deceased; no macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 10y7m - 5m - - Johan den Dunnen 00457866
0000346317 2y3m-deceased; macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; no ataxia; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 2y3m - 7m - - Johan den Dunnen 00457867
0000346318 macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; no ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Unknown 14y - 7y - - Johan den Dunnen 00457868
0000346319 6y11m-deceased; macrocephaly; seizures; no spasticity; no bulbar/pseudobulbar signs; ataxia; cognitive defect; pathology from biopsy; Alexander disease, infantile ALXDRD Unknown 6y11m - 1y6m - - Johan den Dunnen 00457869
0000346320 macrocephaly; seizures; spasticity; no bulbar/pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Unknown 19y - 7m - - Johan den Dunnen 00457870
0000346321 no macrocephaly; seizures; no spasticity; no bulbar/pseudobulbar signs; no ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Unknown 10y - 6y - - Johan den Dunnen 00457871
0000346322 6y5m-deceased; macrocephaly; seizures; spasticity; no bulbar/pseudobulbar signs; no ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Unknown 6y5m - 1d - - Johan den Dunnen 00457872
0000346323 no macrocephaly; no seizures; no spasticity; bulbar and/or pseudobulbar signs; no ataxia; no cognitive defect; MRI brain atypical Alexander disease, juvenile ALXDRD Isolated (sporadic) 17y - 10y - - Johan den Dunnen 00457873
0000346324 <11y6m-chronic bed-wetting; 11y6m-scoliosis; no macrocephaly; no seizures; spasticity; no bulbar/pseudobulbar signs; no ataxia; no cognitive defect; MRI brain atypical Alexander disease, juvenile ALXDRD Isolated (sporadic) 16y - 11y6m - - Johan den Dunnen 00457874
0000346325 33y-deceased; no macrocephaly; no seizures; no spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; pathology from autopsy; MRI brain atypical Alexander disease, adult ALXDRD Unknown 33y - 24y - - Johan den Dunnen 00457875
0000346326 10y-deceased; no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; MRI brain atypical Alexander disease, juvenile ALXDRD Isolated (sporadic) 10y - 3y - - Johan den Dunnen 00457876
0000346327 8y-deceased; no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; pathology from biopsy; MRI brain atypical Alexander disease, juvenile ALXDRD Isolated (sporadic) 8y - 3y - - Johan den Dunnen 00457877
0000346328 macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Isolated (sporadic) 12y - 2y6m - - Johan den Dunnen 00457878
0000346329 no macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Isolated (sporadic) 3y - 2y - - Johan den Dunnen 00457879
0000346330 macrocephaly; seizures; spasticity; no bulbar/pseudobulbar signs; no ataxia; no cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 2y - 1y2m - - Johan den Dunnen 00457880
0000346331 macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 8y - 8m - - Johan den Dunnen 00457881
0000346332 8y-deceased; no macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 8y - 1y6m - - Johan den Dunnen 00457882
0000346333 2y5m-deceased; macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Unknown 2y5m - 1m - - Johan den Dunnen 00457883
0000346334 no macrocephaly; no seizures; no spasticity; no bulbar/pseudobulbar signs; ataxia; cognitive defect; pathology from biopsy; MRI brain atypical Alexander disease, juvenile ALXDRD Unknown 13y - 4y - - Johan den Dunnen 00457884
0000346335 11m15d-deceased; macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Unknown 11m15d - 3m - - Johan den Dunnen 00457885
0000346336 11m15d-deceased; macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Unknown 11m15d - 2m - - Johan den Dunnen 00457886
0000346337 5y3m-deceased; macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Unknown 5y3m - 4m - - Johan den Dunnen 00457887
0000346338 no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 3y - 6m - - Johan den Dunnen 00457888
0000346339 no macrocephaly; seizures; spasticity; no bulbar/pseudobulbar signs; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 6m - 3m - - Johan den Dunnen 00457889
0000346340 macrocephaly; no seizures; no spasticity; bulbar and/or pseudobulbar signs; ataxia; cognitive defect; pathology from biopsy; MRI brain atypical Alexander disease, juvenile ALXDRD Isolated (sporadic) 18y - 2y - - Johan den Dunnen 00457890
0000346341 no macrocephaly; no seizures; no spasticity; bulbar and/or pseudobulbar signs; no ataxia; no cognitive defect; MRI brain atypical Alexander disease, juvenile ALXDRD Unknown 17y - 9y - - Johan den Dunnen 00457891
0000346342 no macrocephaly; no seizures; no spasticity; bulbar and/or pseudobulbar signs; no ataxia; no cognitive defect; MRI brain atypical Alexander disease, infantile ALXDRD Unknown 8y - <1y - - Johan den Dunnen 00457892
0000346343 no macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; no ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Isolated (sporadic) 13y - 5y - - Johan den Dunnen 00457893
0000346344 3m15d-deceased; no macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 3m15d - 1m - - Johan den Dunnen 00457894
0000346345 38d-deceased; macrocephaly (75th percentile), rapidly expanding prior to death; seizures; no spasticity; no bulbar/pseudobulbar signs; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 38d - 1d - - Johan den Dunnen 00457895
0000346346 no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; no ataxia; cognitive defect; MRI brain typical Alexander disease, juvenile ALXDRD Isolated (sporadic) 20y - 3y6m - - Johan den Dunnen 00457896
0000346347 4m-deceased; macrocephaly; seizures; no spasticity; no bulbar/pseudobulbar signs; pathology from autopsy; Alexander disease, infantile ALXDRD Isolated (sporadic) 4m - 1d - - Johan den Dunnen 00457897
0000346348 no macrocephaly; seizures; no spasticity; no bulbar/pseudobulbar signs; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 1y6m - 1m - - Johan den Dunnen 00457898
0000346349 6m-deceased; macrocephaly; seizures; no spasticity; bulbar and/or pseudobulbar signs; cognitive defect; pathology from autopsy; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 6m - 1m15d - - Johan den Dunnen 00457899
0000346350 macrocephaly; bulbar and/or pseudobulbar signs; cognitive defect; pathology from biopsy; MRI brain typical Alexander disease, infantile ALXDRD Unknown 2y - 2m - - Johan den Dunnen 00457900
0000346351 macrocephaly; seizures; no spasticity; no bulbar/pseudobulbar signs; no ataxia; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 6m - 3m - - Johan den Dunnen 00457901
0000346352 no macrocephaly; seizures; spasticity; no bulbar/pseudobulbar signs; ataxia; cognitive defect; MRI brain typical Alexander disease, infantile ALXDRD Isolated (sporadic) 4y - 1y6m - - Johan den Dunnen 00457902
0000346353 no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; pathology from biopsy; MRI brain typical Alexander disease, juvenile ALXDRD Unknown 25y - 10y - - Johan den Dunnen 00457903
0000346354 8m-deceased; no macrocephaly; seizures; spasticity; bulbar and/or pseudobulbar signs; cognitive defect; MRI brain atypical Alexander disease, infantile ALXDRD Unknown 8m - <7d - - Johan den Dunnen 00457904
0000346355 59y-deceased; no macrocephaly; no seizures; spasticity; bulbar and/or pseudobulbar signs; ataxia; no cognitive defect; pathology from autopsy; MRI brain atypical Alexander disease, adult ALXDRD Unknown 59y - 45y - - Johan den Dunnen 00457905
0000346356 no macrocephaly; no seizures; no spasticity; no bulbar/pseudobulbar signs; ataxia; cognitive defect; MRI brain typical with additional increased signal intensity bilaterally within cerebral peduncles, crossing fibers pons, dentate nuclei Alexander disease, juvenile ALXDRD Unknown 13y9m - 9y - - Johan den Dunnen 00457906
0000346395 see paper; ..., 13y-slowly progressive motor/mental deterioration, slurred nasal voice, elevation αB-crystallin/HSP27 in CSF, characteristic neuroimaging findings (frontal dominant leukodystrophy with cyst formation, prominent involvement arcuate fibers, diffuse symmetric lesions basal nuclei, subependymal stripes around ateral ventricle); 8y-generalized febrile convulsion with bilateral frontal spikes, school performance declined, motor function worsened; 21y-walk short distances independently, speak slowly in simple sentences, play video games juvenile Alexander disease ALXDRD Isolated (sporadic) 21y - - - - Johan den Dunnen 00457945
0000346398 see paper; ..., 38d deceased (prolonged seizures, respiratory failure); 1d-poor feeding; 5d-emesis, poor feeding infantile Alexander disease ALXDRD Unknown 00y00m38d - - - - Johan den Dunnen 00457948
0000346399 see paper; ..., bulbar signs, sleep disturbance (mainly sleep apnea), symptoms of dysautonomia, dysmorphism; MRI brain younger patients T2 signal abnormalities medulla (area of demyelination), older patients marked atrophy medulla, no signal abnormalities adult Alexander's disease ALXDRD Familial, autosomal dominant - - - - - Johan den Dunnen 00457949
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