Hereditary and idiopathic spastic paraparesis: preliminary findings of a single center experience

Neurol Res. 2018 Dec;40(12):1088-1093. doi: 10.1080/01616412.2018.1522412.


Objectives: Hereditary spastic paraplegias (HSP) is a heterogeneous group of inherited neurologic disorders with diversified clinical manifestations. The purpose of this study was to summarize the clinical manifestations of HSP by analyzing the clinical data of 56 HSP patients.

Methods: A total of 56 HSP patients treated in our hospital from January 2014 to March 2016 were included. Demographic and clinical characteristics of patients were collected. The severity of HSP was assessed by disease severity score.

Results: The patients included 40 males and 16 females. The mean onset age was 17.86 ± 12.56 years (range: 1-47). The mean disease duration was 13.46 ± 12.82 years (range: 1-63). There were 29 pure (51.8%) forms and 27 complicated (48.2%) HSP. The common manifestations included increased deep tendon reflexes in the lower extremities (94.6%), positive Babinski sign (94.6%), increased muscle tone of lower extremities (91.1%), scissors gait (83.9%), ankle clonus (69.6%), reduced muscle strength in the lower extremities (48.2%) and skeletal deformities (37.5%). Reduced cognitive function was the most common manifestation (55.6%) of the complicated HSP patients. The mean disease severity score was significantly higher in males than in females (2.75 ± 0.55 vs. 2.18 ± 1.13, P = 0.013). Patients with a disease duration >30 years had a significantly higher disease severity score than those with disease duration of 1-10 and 21-30 years.

Discussion: We reported the clinical features of HSP from 56 patients in our hospital. Our findings should be helpful for better understanding of clinical features of HSP.

Keywords: Hereditary spastic paraplegias (HSP); cognitive function; complicated hereditary spastic paraplegias (HSP); pure hereditary spastic paraplegias (HSP).

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cognition Disorders / diagnosis
  • Cognition Disorders / etiology*
  • Female
  • Humans
  • Infant
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Retrospective Studies
  • Severity of Illness Index
  • Spastic Paraplegia, Hereditary / complications*
  • Spastic Paraplegia, Hereditary / diagnostic imaging
  • Statistics, Nonparametric
  • Tomography Scanners, X-Ray Computed
  • Young Adult