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Case Report
Pseudopolyneuritic form of amyotrophic lateral sclerosis: Marie-Patrikios type
1 Master's Program in Neurology - University of Vassouras and School of Medicine - University Iguaçu - RJ, Brazil
2 Iguaçu University - UNIG - Department of Neurology of Hospital Geral de Nova Iguaçu - RJ, Brazil
3 Department of Neurology of Federal University of Rio de Janeiro - UFRJ, Brazil
4 Department of Medicine - UNIFESP - Paulista School of Medicine, Brazil
Address correspondence to:
Antônio Marcos da Silva Catharino
Rua Gavião Peixoto 70, Room 811, CEP 24.2230-100, Icaraí, Niterói, RJ,
Brazil
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Article ID: 101340Z01MO2022
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Orsini M, da Silva Catharino AM, Silveira VC, Reis CHM, de Freitas MRG, de Oliveira AB. Pseudopolyneuritic form of amyotrophic lateral sclerosis: Marie-Patrikios type. Int J Case Rep Images 2022;13(2):118–121.ABSTRACT
Introduction: Amyotrophic lateral sclerosis (ALS), also called motor neuron disease (MND), is a progressive, neurodegenerative, and inexorable disease that affects the neurons of the anterior horn of the spinal cord, as well as the lateral funiculus. A rare variant of ALS was first described in 1918 by Patrikios and Marie, called the pseudopolyneuritic form or Marie-Patrikios disease. It is characterized by an initial manifestation with melting of the feet, distal weakness of the muscles of the anterior compartment of the leg, and absence of the Achilles tendon reflex. We present an atypical case of ALS, marked by polyneuropathy and involvement of upper and lower motor neurons.
Case Report: A 70-year-old man reported that approximately four years ago he started having pain in the thoracic region with subsequent paresis in the lower limbs. Initially, compressive myelopathy, transverse myelitis, and spastic paraparesis of various causes were thought to be the cause. However, the non-impairment of the superficial and deep sensibility, obviously, with absence of sensorial level, associated to the absence of specific imaging findings in the thoracic and lumbar spine, a normal complete laboratory, ruled out such hypotheses.
Conclusion: We highlight that the pseudopolyneuritic form presented in this study has a better prognosis and survival rate when compared to other subtypes of ALS. Thus, a detailed investigation including physical, neurological, and electrophysiological examination is essential to establish the diagnosis and increase the scarce knowledge about this condition.
Keywords: Achilles tendon reflex, Amyotrophic lateral sclerosis, Heterogeneity, Pseudopolyneuritic form
SUPPORTING INFORMATION
Author Contributions
Marco Orsini - Substantial contributions to conception and design, Acquisition of data, Drafting the article, Final approval of the version to be published
Antônio Marcos da Silva Catharino - Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Valéria Camargo Silveira - Analysis of data, Revising it critically for important intellectual content, Final approval of the version to be published
Carlos Henrique Melo Reis - Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Marcos RG de Freitas - Substantial contributions to conception and design, Analysis of data, Interpretation of data, Revising it critically for important intellectual content, Final approval of the version to be published
Acary Bulle de Oliveira - Acquisition of data, Analysis of data, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2022 Marco Orsini et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
