Autosomal recessive spastic paraplegia type 20

Orpha code: 101000OMIM code: 275900

Definition

Autosomal recessive spastic paraplegia type 20 (SPG20) is a type of complex hereditary spastic paraplegia characterized by an onset in infancy of progressive spastic paraparesis associated with distal amyotrophy, psuedobulbar palsy, motor and cognitive delays, mild cerebellar signs (dysarthria, dysdiadochokinesia, mild intention tremor), short stature and subtle skeletal abnormalities (pes cavus, mild talipes equinovarus, kyphoscoliosis). SPG20 is due to mutations in the <i>SPG20</i> gene (13q13.1), which encodes the protein spartin.

Disease data
Classification

Disease

Synonyms
Childhood-onset spastic paraparesis-distal muscle wasting syndrome
Paraplegia spastyczna o początku w wieku dziecięcym - zanik mięsni dystalnych
SPG20
Zespół Troyera
SPG20
Troyer syndrome
ORPHA code
101000
OMIM code
275900
ICD10 code
G11.4
ICD11 code
-

No additional description.

Orphanet - interntowa baza danych dotyczących rzadkich chorób i sierochych leków. ©INSERM 1999 - Dostępna na stronie www.orphanet.pl