Phenotypes for disease #03514 (EPM6 (epilepsy, myoclonic, progressive, type 6 (EPM-6)), OMIM:614018)

13 entries on 1 page. Showing entries 1 - 13.
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0000041542 7y tremor; 7-8y absences; 8y obvious myoclonus; 13y drop attacks; 13y convulsive seizures; 2y areflexia; 14y wheelchair; 22y bedfast; normal cognition until 25y, mmemory difficulties later; scoliosis, pes cavus; EEG generalized spike-wave, posterior emphasis photosensitive; CK 570–800 (normal <170) - - Isolated (sporadic) - - 2y ataxia - Johan den Dunnen 00054881
0000041543 4y tremor; 6y myoclonus; 14y drop attacks; myoclonic status; 2y areflexia; 15y wheelchair; normal cognition; scoliosis; EEG generalized spike-wave, posterior emphasis photosensitive; CK 150–580 (normal <170) - - Isolated (sporadic) - - 1y6m ataxia - Johan den Dunnen 00054882
0000041544 6y myoclonus; 14y convulsive seizures; 3y areflexia; 13y wheelchair; normal cognition; scoliosis, syndactly; EEG generalized spike-wave photosensitive; CK 141–267 (normal <170) - - Isolated (sporadic) - - 3y ataxia - Johan den Dunnen 00054883
0000041545 5y fine motor problems; 7y worsening ataxia; 6y absences; 10y myoclonus; 12y tonic-clonic; 14y drop attacks; 7y areflexia; 24y wheelchair; normal cognition until 25y, memory difficulties later; scoliosis, syndactly; EEG generalized spike-wave, posterior emphasis photosensitive; CK 700–900 (normal <170) - - Isolated (sporadic) - - 2y acute ataxia, improved - Johan den Dunnen 00054884
0000041546 6y myoclonus; 21y tonic-clonic seizures; 6y areflexia; 14y wheelchair; normal cognition; scoliosis; EEG generalized discharges photosensitive; CK 300–668 (normal <170) - - Isolated (sporadic) - - 2y6m ataxia - Johan den Dunnen 00054885
0000041547 5.5y myoclonus; 24y tonic-clonic seizures; absences; tonic seizures; 3y areflexia; 13y wheelchair; normal cognition; scoliosis; EEG generalized discharges photosensitive; CK 174–213 (normal <170) - - Isolated (sporadic) - - 2y6m ataxia - Johan den Dunnen 00054886
0000041548 Ramsay Hunt Syndrome; 3y ataxia, 5y myoclonus, 9y tonic seizures, 3y areflexia, scoliosis; EEG generalized epileptic discharges, photoconvulsive response; EMG findings indicating sensory neuronopathy, findings indicating anterior horn cell involvement; CK normal; 5y muscle histology normal; 19y ambulant; normal cognition - - Isolated (sporadic) - - 2y gait disorder, clumsiness - Johan den Dunnen 00054887
0000041549 Ramsay Hunt Syndrome; 3y ataxia, 6y myoclonus, 11y generalized tonic clonic seizures, 6y areflexia (not tested earlier), no skeletal abnormaliatie;, EEG generalized epileptic discharges, photoconvulsive response; EMG findings indicating sensory neuronopathy, findings indicating anterior horn cell involvement; CK normal; 19y ambulant; mild learning difficulties - - Isolated (sporadic) - - 2y gait disorder, clumsiness - Johan den Dunnen 00054888
0000041550 Ramsay Hunt Syndrome; 2y ataxia, 6y myoclonus, 6y generalized tonic clonic seizures, 9y areflexia (not tested earlier), scoliosis, syndactyl;, EEG generalized epileptic discharges, photoconvulsive response; EMG findings indicating sensory neuronopathy; CK normal; 8y wheelchair-bound; mild learning difficulties - - Isolated (sporadic) - - 2y gait disorder, clumsiness - Johan den Dunnen 00054889
0000041551 Ramsay Hunt Syndrome; 5y ataxia, 8y myoclonus, 3y clonic seizures, 5y areflexia, scoliosis,; EEG generalized epileptic discharges, photoconvulsive response; EMG findings indicating sensory neuronopathy, findings indicating anterior horn cell involvement; CK 400-500 (normal <200); 7y/10y muscle histology normal; 12y ambulant; normal cognition - - Isolated (sporadic) - - 3y febrile seizures - Johan den Dunnen 00054890
0000041552 2y-frequent falls, clumsy gait; 3y ataxia, 6y myoclonus, no seizures, 3y areflexia, no skeletal abnormalities; 3y EMG normal; CK normal; 7y ambulant; normal cognition 2y-frequent falls, clumsy gait EPM6 Isolated (sporadic) - - 02y gait disorder, clumsiness - Johan den Dunnen 00054891
0000127062 - - - Familial, autosomal recessive - - - - - Elizabeth Ulm 00154385
0000257411 Onset age 14 of ataxia. Severe myoclonus from age 32, TCS, moderate dementia. Dysphagia and pyramidal signs noted. Psychiatric co-morbidities. Severe cerebral, moderate cerebellar atrophy. EEG: photoparoxysmal response. progressive myoclonus epilepsy , dementia - Familial, autosomal recessive - - - - - Johan den Dunnen 00334904
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