ICD-11 classes
08 Diseases of the nervous system
Spinal cord disorders excluding trauma
8B44 — Degenerative myelopathic disorders
8B44.0 — Hereditary spastic paraplegia

ICD-11 8B44.0 — Hereditary spastic paraplegia

Hereditary spastic paraplegias (HSP) comprise a genetically and clinically heterogeneous group of neurodegenerative disorders characterised 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, ichthyosis and extrapyramidal involvement, in addition to spastic paraparesis. Pure HSPs are characterised 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 characterised 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.

The diagnosis includes nothing.

The diagnosis excludes nothing.

Diagnosis with code 8B44.0 contains 5 clarifying diagnoses:

1. 8B44.00 — Autosomal dominant hereditary spastic paraplegia
2. 8B44.01 — Autosomal recessive hereditary spastic paraplegia
3. 8B44.02 — X-linked hereditary spastic paraplegia
4. 8B44.0Y — Other specified hereditary spastic paraplegia
5. 8B44.0Z — Hereditary spastic paraplegia, unspecified

The diagnosis is coded elsewhere:

• Certain specified movement disorder #6725
• Hereditary spastic paraplegia #6755