Description of the disease * EnglishPolish Pobierz sekcję do PDF Definicja Spastic paraplegia-severe developmental delay-epilepsy syndrome is a rare, genetic, complex spastic paraplegia disorder characterized by an infantile-onset of psychomotor developmental delay with severe intellectual disability and poor speech acquisition, associated with seizures (mostly myoclonic), muscular hypotonia which may be noted at birth, and slowly progressive spasticity in the lower limbs leading to severe gait disturbances. Ocular abnormalities and incontinence are commonly associated. Other symptoms may include verbal dyspraxia, hypogenitalism, macrocephaly and sensorineural hearing loss, as well as dystonic movements and ataxia with upper limb involvement. Disease data Klasyfikacja Disease Synonimy SPPRS syndrome Zespół SPPRS Zespół paraplegii spastycznej, opóźnienia psychoruchowego i napadów padaczkowych Spastic paraplegia-psychomotor retardation-seizures syndrome Kod ORPHA 464282 Kod OMIM 616756 Kod ICD10 - Kod ICD11 - *Soruce Extended description of the disease Pobierz sekcję do PDF No additional description. Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl