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Spinal cord stimulation may improve gait and cognition in hereditary spastic paraplegia with mental retardation: a case report

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Abstract

Background

Hereditary spastic paraplegia (HSP) include various sporadic and hereditary neurodegenerative disorders, characterized by progressive spasticity and weakness of lower limbs, possibly associated to additional features.

Case presentation

We report a male HPS patient in his 40 s, showing mental retardation associated with language impairment, dysarthria, and increased urinary frequency. Three months after treatment with electric chronic high-frequency cervical spinal cord stimulation (HF-SCS), he showed an amelioration of motor symptoms (lower limbs spasticity and gait), dysarthria, cognitive functioning (language and constructive praxic abilities), and urinary symptoms (decreased urinary frequency). Single-photon emission computed tomography (SPECT) showed a postoperative increase of cerebral perfusion in right frontal cortex and temporal cortex bilaterally.

Conclusion

In our patient, HF-SCS might have induced an activation of ascending neural pathways, resulting in changes in activity in various cortical areas (including sensory-motor cortical areas), which may give rise to a modulation of activity in spared descending motor pathways and in neural networks involved in cognitive functions, including language. Although further studies in patients with HPS are needed to clarify whether HF-SCS can be a suitable treatment option in HSP, our observation suggests that HF-SCS, a minimally invasive neurosurgical procedure, might induce beneficial effects of on various symptoms of such orphan disease.

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Authors and Affiliations

  1. Neurosurgery Unit, IRCCS Fondazione Policlinico Universitario A.Gemelli, Rome, Italy

    Tommaso Tufo

  2. IRCCS Neuromed, Pozzilli, IS, Italy

    Marco Ciavarro

  3. Nuclear Medicine Unit, IRCCS Fondazione Policlinico Universitario A. Gemelli, Rome, Italy

    Daniela Di Giuda

  4. Department of Radiological and Hematological Sciences, Università Cattolica del Sacro Cuore, Rome, Italy

    Daniela Di Giuda

  5. Department of Geriatrics, Neurosciences and Orthopedics, High Intensity Neurorehabilitation Unit, Policlinico Universitario A. Gemelli, Rome, Italy

    Chiara Piccininni

  6. Department of Neuroscience, Università Cattolica del Sacro Cuore, Rome, Italy

    Carla Piano & Antonio Daniele

  7. Neurology Unit, IRCCS Fondazione Policlinico Universitario A. Gemelli, Rome, Italy

    Antonio Daniele

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  1. Tommaso Tufo
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  2. Marco Ciavarro
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  3. Daniela Di Giuda
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  4. Chiara Piccininni
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  5. Carla Piano
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  6. Antonio Daniele
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Correspondence to Marco Ciavarro.

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Tufo, T., Ciavarro, M., Di Giuda, D. et al. Spinal cord stimulation may improve gait and cognition in hereditary spastic paraplegia with mental retardation: a case report. Neurol Sci 44, 961–966 (2023). https://doi.org/10.1007/s10072-022-06487-w

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  • DOI: https://doi.org/10.1007/s10072-022-06487-w

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