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Diabetic Neuropathy01:22

Diabetic Neuropathy

DefinitionDiabetic neuropathy is nerve damage caused by long-standing diabetes mellitus. It results directly from prolonged high blood sugar levels.PathophysiologyThe pathophysiology of diabetic neuropathy involves both metabolic and vascular disturbances triggered by chronic hyperglycemia.Metabolic injury: Elevated glucose levels activate the polyol pathway within nerve cells, leading to the accumulation of sorbitol and fructose. This increases oxidative stress, disrupts normal nerve...
Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
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Digestion begins with a cephalic phase that prepares the digestive system to receive food. When our brain processes visual or olfactory information about food, it triggers impulses in the cranial nerves innervating the salivary glands and stomach to prepare for food.
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Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
Diabetic Foot Ulcer01:31

Diabetic Foot Ulcer

Definition A diabetic foot ulcer (DFU) is a chronic, non-healing wound that develops in individuals with diabetes. It typically occurs on pressure-bearing areas such as the heel, metatarsal heads, or hallux, and carries a high risk of infection and amputation.Pathophysiology • The development of DFUs can be explained by four interconnected mechanisms: neuropathy, ischemia, infection, and impaired wound healing. • Neuropathy is the most common factor. Sensory neuropathy reduces pain perception,...
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Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...

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Updated: Jun 8, 2026

Nerve Ultrasound Protocol to Detect Dysimmune Neuropathies
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Neuropathy in Wolfram syndrome.

Stéphane Mathis1, Thierry Maisonobe, Jean-Philippe Neau

  • 1Université de Poitiers, CHU de Poitiers, Clinique Neurologique, 2 rue de la Milétrie, 86021 Poitiers Cedex 05, France. s.mathis@chu-poitiers.fr

European Journal of Medical Genetics
|October 5, 2010
PubMed
Summary

Wolfram syndrome (WFS) patients rarely exhibit polyneuropathy. When present, EMG studies reveal an axonal sensory-motor polyneuropathy, potentially linked to diabetes or WFS itself.

Area of Science:

  • Neurology
  • Endocrinology
  • Genetics

Background:

  • Wolfram syndrome (WFS) is a rare, inherited neurodegenerative disorder.
  • It is characterized by diabetes mellitus and optic atrophy.
  • Neurological and endocrine dysfunctions are hallmarks of WFS.

Observation:

  • Polyneuropathy is an exceptionally reported symptom in Wolfram syndrome.
  • This study reviewed over 600 case reports and selected 21 patients who underwent EMG.
  • EMG findings were abnormal in only 8 of these patients.

Findings:

  • The predominant EMG profile in affected patients was axonal sensory-motor polyneuropathy.
  • Some cases showed a significant reduction in motor nerve conduction velocities.
  • The neuropathy's origin remains unclear, possibly related to diabetes or the underlying WFS pathology.

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Implications:

  • This research clarifies the characteristics of polyneuropathy in Wolfram syndrome.
  • Understanding this association aids in diagnosing and managing WFS patients.
  • Further investigation into the neuropathic mechanisms in WFS is warranted.