Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Comment
. 2018 Mar;28(2):292-294.
doi: 10.1111/bpa.12494.

Hereditary spastic paraplegia type 8: Neuropathological findings

Caroline Pehrson  1 , Jens Michael Hertz  2   3 , Martin Wirenfeldt  1   2 , Egon Stenager  4 , Lene Wermuth  5 , Bjarne Winther Kristensen  1   2
Affiliations
Comment

Hereditary spastic paraplegia type 8: Neuropathological findings

Caroline Pehrson et al. Brain Pathol. 2018 Mar.
No abstract available

PubMed Disclaimer

Conflict of interest statement

The studies in this report did not receive separate funding, and the authors declare no conflicts of interest.

Figures

Figure 1
Figure 1
Macroscopic appearance of the spinal cord. A. Spinal cord with severe atrophy. B. The portion between the cervical and lumbar enlargements consists mainly of meninges and vessels. C. Cervical enlargement. D. Lumbar enlargement.
Figure 2
Figure 2
Histological appearance of the medulla oblongata and spinal cord. A. Medulla oblongata. B, C. Cervical enlargement with reduction in number of axons as demonstrated at high magnification of a luxol fast blue stained slide (insert F). D. Severely atrophied spinal cord between the cervical and lumbar enlargements with marked reduction in number of axons. E. Lumbar enlargement with disorganized overall histology and reduction in number of axons demonstrated at high magnification of a luxol fast blue stained slide (insert G). Bars: A, B – 5 mm; C–E – 2.5 mm; F, G – 250 µm.
See this image and copyright information in PMC

Comment on

References

    1. Behan WM, Maia M (1974) Strumpell's familial spastic paraplegia: genetics and neuropathology. J Neurol Neurosurg Psychiatry 37:8–20. - PMC - PubMed
    1. Bettencourt C, Lopez‐Sendon JL, Garcia‐Caldentey J, Rizzu P, Bakker IM, Shomroni O et al (2014) Exome sequencing is a useful diagnostic tool for complicated forms of hereditary spastic paraplegia. Clin Genet 85:154–158. - PubMed
    1. Clemen CS, Tangavelou K, Strucksberg KH, Just S, Gaertner L, Regus‐Leidig H et al (2010) Strumpellin is a novel valosin‐containing protein binding partner linking hereditary spastic paraplegia to protein aggregation diseases. Brain 133:2920–2941. - PubMed
    1. de Bot ST, Schelhaas HJ, Kamsteeg EJ, van de Warrenburg BP (2012) Hereditary spastic paraplegia caused by a mutation in the VCP gene. Brain 135:1–3. - PubMed
    1. de Bot ST, Vermeer S, Buijsman W, Heister A, Voorendt M, Verrips A et al (2013) Pure adult‐onset Spastic Paraplegia caused by a novel mutation in the KIAA0196 (SPG8) gene. J Neurol 260:1765–1769. - PubMed