Forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis, or motor neuron disease with pallido-nigro-luysian atrophy? An autopsy case report

Neuropathology : Official Journal of the Japanese Society of Neuropathology
Tomoyo HashimotoKiyomitsu Oyanagi

Abstract

We describe a 52-year-old man with body weight loss and bulbar palsy, who exhibited muscle atrophy and weakness with fasciculation especially in the respiratory muscles 4 years prior to death, necessitating respiratory support for 4 years, but who was able to walk until the end-stage. He had no significant family history. Neuropathological examination revealed severe loss of motor neurons in the spinal cord and brainstem, and ubiquitin-positive skein-like inclusions and Bunina bodies in the remaining neurons. In addition, prominent degeneration of the anterolateral funiculus and severe loss of neurons in the intermediate zone of the spinal cord were evident, without marked alteration of the corticospinal tracts. Degeneration of the subthalamic nucleus, increased iron deposition in the substantia nigra, and axonal swelling, residual nodules and acidophilic granules in the spinal ganglia were found. The patient's condition was considered to have been a forme fruste or incipient form of widespread-type amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) with pallido-nigro-luysian atrophy (PNLA). The neuropathological features of the present case appear to be important for understanding the nature of widespread-type A...Continue Reading

References

Apr 1, 1992·Journal of the Neurological Sciences·K HasegawaS Yagishita
Jan 1, 1992·Acta Neuropathologica·S SasakiS Maruyama
Nov 1, 1991·Journal of Neurochemistry·D Ben-ShacharM B Youdim
Oct 1, 1989·Journal of the Neurological Sciences·H Hayashi, S Kato
Nov 11, 1988·Neuroscience Letters·P N LeighD M Power
Jan 1, 1985·Acta Neuropathologica·F GrayJ Poirier
Jan 1, 1981·Acta Neuropathologica. Supplementum·F GrayR Escourolle
Jan 1, 1993·Acta Neuropathologica·M BergmannF Gullotta

❮ Previous
Next ❯

Citations

Aug 26, 2009·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Yasuo MikiKoichi Wakabayashi
Nov 2, 2012·Movement Disorders : Official Journal of the Movement Disorder Society·Janice C WongLili-Naz Hazrati
May 23, 2013·Respiratory Physiology & Neurobiology·Nicole L NicholsG S Mitchell
Jul 1, 2008·Advances in Virus Research·Neal Nathanson
Sep 15, 2017·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Akiko UchinoShigeo Murayama
Dec 3, 2016·Journal of Neuromuscular Diseases·Annerieke C van GroenestijnJohanna M A Visser-Meily

❮ Previous
Next ❯

Related Concepts

Related Feeds

ALS: Stress Granules

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by cytoplasmic protein aggregates within motor neurons. TDP-43 is an ALS-linked protein that is known to regulate splicing and storage of specific mRNAs into stress granules, which have been implicated in formation of ALS protein aggregates. Here is the latest research in this field.

Basal Ganglia

Basal Ganglia are a group of subcortical nuclei in the brain associated with control of voluntary motor movements, procedural and habit learning, emotion, and cognition. Here is the latest research.

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.