Few studies focused on the long-term outcome of @entity3148 in adulthood are available in the literature, but all are concordant. In this article, we consider the outcomes of 24 @entity1 followed at the Centre Saint-Paul, Marseille, up to the age of 50, and compare them to the @entity1 reported in the literature. Five @entity1 (20.8%) died, at a mean age of 24.8 years, one by @entity159 , three by @entity944 ( @entity944 ), and one of unknown cause. @entity302 tend to become less frequent and less severe after childhood. Fever sensitivity (temperature variations) persists throughout the clinical course of DS, but its impact on @entity410 frequency and severity is milder than in infancy. Generalized @entity410 , mostly reported as generalized @entity410 (GTCS), were the only @entity410 type observed in almost all of the @entity1 , often with a focal onset. They are less frequent than in childhood and mostly nocturnal. Some of these major @entity410 have less typical aspects, for example, bilateral or asymmetric tonic posturing, followed in some cases by a tonic vibratory state or clonic movements (Oguni et al., Brain Dev 2001;23:736-748; Akiyama et al., @entity10895 2010;51:1043-1052). Other @entity410 like @entity410 , atypical absences, and @entity410 ( @entity410 ) are less common in adulthood: Among our 24 @entity1 , only 6 had atypical absences, and one @entity410 . Electroencephalography (EEG) also changes with age but is still multiple and heterogenous, interictally and ictally. Photosensitivity and pattern sensitivity also showed a tendency to disappear before the age of 20. @entity4681 are common. Cerebellar features, @entity2055 , @entity4653 , intention @entity400 , and @entity1319 , become more prominent. Walking is markedly impaired, often due to orthopedic signs such as @entity1513 , @entity3792 , flat feet, or claw feet. This symptomatology was minor during childhood and worsened during and after adolescence, despite physiotherapy. @entity1032 ranged from moderate to severe, with predominance of @entity73 , and some @entity1 had a major @entity1314 , labeled autistic or @entity716 . Dependency in adulthood is nearly constant: Only 3 of our 24 adult @entity1 lived independently.

XXXX : the long-term outcome.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity410 :: ('MESH:D012640', 'Disease') :: ['seizure', 'complex partial seizures', 'tonic-clonic seizures', 'CPS', 'myoclonic seizures', 'convulsive seizures', 'seizures', 'myoclonic and one complex focal seizures'] @entity1032 :: ('MESH:D008607', 'Disease') :: ['Mental retardation'] @entity944 :: ('MESH:D003645', 'Disease') :: ['SUDEP', 'sudden unexpected death in epilepsy'] @entity73 :: ('MESH:D003072', 'Disease') :: ['language impairment'] @entity1513 :: ('MESH:D007738', 'Disease') :: ['kyphosis'] @entity10895 :: ('MESH:D017036', 'Disease') :: ['Epilepsia'] @entity4681 :: ('MESH:D016472', 'Disease') :: ['Motor abnormalities'] @entity1319 :: ('MESH:D015835', 'Disease') :: ['eye movement disorder'] @entity3792 :: ('MESH:C565711', 'Disease') :: ['kyphoscoliosis'] @entity400 :: ('MESH:D014202', 'Disease') :: ['tremor'] @entity302 :: ('MESH:D004827', 'Disease') :: ['Epileptic seizures'] @entity716 :: ('MESH:D011605', 'Disease') :: ['psychotic'] @entity3148 :: ('MESH:D004831', 'Disease') :: ['Dravet syndrome'] @entity1314 :: ('MESH:D010554', 'Disease') :: ['personality disorder'] @entity159 :: ('MESH:D013226', 'Disease') :: ['status epilepticus'] @entity2055 :: ('MESH:D001259', 'Disease') :: ['including ataxia'] @entity4653 :: ('MESH:D004401', 'Disease') :: ['dysarthria']
@entity3148 :: (MESH:D004831,Disease) :: ['Dravet syndrome']
