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Autosomal recessive spastic paraplegia type 9B

ORPHA:447760DiseaseAutosomal recessiveChildhood, Infancy

Ассоциированные гены (1)

ALDH18A1
aldehyde dehydrogenase 18 family member A1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 138250

Фенотипы (29)

Очень частый (80–99%)4
HP:0001257Spasticity
HP:0002395Lower limb hyperreflexia
HP:0003487Babinski sign
HP:0007350Hyperreflexia in upper limbs
Частый (30–79%)8
HP:0001260Dysarthria
HP:0001270Motor delay
HP:0001324Muscle weakness
HP:0001510Growth delay
HP:0002064Spastic gait
HP:0002174Postural tremor
HP:0002445Tetraplegia
HP:0031064Impaired continence
Периодический (5–29%)17
HP:0000016Urinary retention
HP:0000252Microcephaly
HP:0000750Delayed speech and language development
HP:0001263Global developmental delay
HP:0001999Abnormal facial shape
HP:0002120Cerebral cortical atrophy
HP:0002371Loss of speech
HP:0002476Primitive reflex
HP:0002518Abnormal periventricular white matter morphology
HP:0002751Kyphoscoliosis
HP:0003202Skeletal muscle atrophy
HP:0003438Absent Achilles reflex
HP:0004322Short stature
HP:0006938Impaired vibration sensation at ankles
HP:0007371Corpus callosum atrophy
HP:0030051Tip-toe gait
HP:0100515Pollakisuria

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы