ICD-11 code : 8B44.0;
Preferred Label : Hereditary spastic paraplegia;
ICD-11 definition : Hereditary spastic paraplegias (HSP) comprise a genetically and clinically heterogeneous
group of neurodegenerative disorders characterized by varying degrees of lower limb
spasticity, pyramidal weakness, hyperreflexia and hypertonic bladder involvement.
Clinically, HSPs can be divided into two main groups: uncomplicated (pure) and complicated
(complex) forms depending on the presence of other neurological features including
ataxia, peripheral neuropathy, cognitive impairment, epilepsy, amyotrophy, retinopathy,
deafness, icthyosis and extrapyramidal involvement, in addition to spastic paraparesis.
Pure HSPs are characterized by slowly progressive lower extremity spasticity and weakness,
often associated with hypertonic urinary disturbances, mild reduction of lower extremity
vibration sense and, occasionally, of joint position sensation. Complex HSP forms
are characterized by the presence of additional neurological or non-neurological features.
A positive family history particularly in autosomal dominant cases is often but not
always present. The diagnosis may be aided by neuroimaging and genetic testing.;
ICD-11 synonym : Spastic paraparesis; hereditary spastic paralysis; Familial spastic paraplegia; Strümpell-Lorrain disease;
ICD-11 inclusion : familial spastic paralysis; hereditary spastic ataxia;
ICD-11 "other" category code : 8B44.0Y;
ICD-11 "unspecified" category code : 8B44.0Z;
Origin ID : 810807375;
UMLS CUI : C0037773;
Currated CISMeF NLP mapping
- ICD-10 Mapping
- Semantic type(s)
- UMLS correspondences (same concept)
- Validated automatic mappings to NTBT
Hereditary spastic paraplegias (HSP) comprise a genetically and clinically heterogeneous
group of neurodegenerative disorders characterized by varying degrees of lower limb
spasticity, pyramidal weakness, hyperreflexia and hypertonic bladder involvement.
Clinically, HSPs can be divided into two main groups: uncomplicated (pure) and complicated
(complex) forms depending on the presence of other neurological features including
ataxia, peripheral neuropathy, cognitive impairment, epilepsy, amyotrophy, retinopathy,
deafness, icthyosis and extrapyramidal involvement, in addition to spastic paraparesis.
Pure HSPs are characterized by slowly progressive lower extremity spasticity and weakness,
often associated with hypertonic urinary disturbances, mild reduction of lower extremity
vibration sense and, occasionally, of joint position sensation. Complex HSP forms
are characterized by the presence of additional neurological or non-neurological features.
A positive family history particularly in autosomal dominant cases is often but not
always present. The diagnosis may be aided by neuroimaging and genetic testing.
