Spastic paraplegia-severe developmental delay-epilepsy syndrome

Orpha code: 464282OMIM code: 616756

Definition

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
Classification

Disease

Synonyms
SPPRS syndrome
Zespół SPPRS
Zespół paraplegii spastycznej, opóźnienia psychoruchowego i napadów padaczkowych
Spastic paraplegia-psychomotor retardation-seizures syndrome
ORPHA code
464282
OMIM code
616756
ICD10 code
-
ICD11 code
-

No additional description.

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