What is Neuropathy due to Inherited Metabolic Diseases?

Neuropathy due to Inherited Metabolic Diseases
Neuropathy due to Inherited Metabolic Diseases. Normal and basic pathological nerve biopsy findings. A: Sural nerve fascicle of a 65-year-old patient showing normal density and distribution of large and small myelinated nerve fibers. Semithin section, toluidine blue. Scale bar = 80 µm. B: Diabetic neuropathy: Axonal neuropathy pattern with reduced myelinated nerve fiber density and clusters of regenerating nerve fibers (black arrows). Marked thickening of endoneurial blood vessel walls (white arrows) due to basal lamina reduplication and widening. Semithin section, toluidine blue. Scale bar = 50 µm. C: Rapidly progressive axonal neuropathy associated with acute nerve fiber breakdown. Arrows: macrophages containing myelin debris. Semithin section, toluidine blue. Scale bar = 15 µm. D: Demyelinating neuropathy (CMT1A). Schwann cell processes are forming onion bulbs around a myelinated (black arrow) and a non-myelinated axon. Semithin section, toluidine blue. Scale bar = 15 µm. E: Normal ultrastructural appearance of large and small myeunated, and unmyelinated (black arrows) nerve fibers. White arrows: retraction of the axon from the inner mesaxon, a frequent artifact. EM. Scale bar = 3 µm. F: Loss of unmyelinated axons (A) associated with numerous Schwann cell processes ensheathing bundles of collagen fibers, so-called empty collagen pockets. N = Schwann cell nucleus. EM. Scale bar = 2 µm. Processing of nerve biopsies: a practical guide for neuropathologists: Weis J, Brandner S, Lammens M, Sommer C, Vallat JM - Clinical neuropathology (2012 Jan-Feb). Not altered. CC.

Neuropathy due to Inherited metabolic diseases is the disease of the nervous system caused by a chemical disruption in the body that leads to the alteration of the structure and the function of the myelin and the axons.

What is the Pathology of Neuropathy due to Inherited Metabolic Diseases?

The pathology of neuropathy due to inherited metabolic diseases is:

-Etiology: The cause of neuropathy due to inherited metabolic diseases is inherited disorders such as porphyria, uremia, and diabetes.

-Genes involved: NA.

-Pathogenesis: The sequence of events that lead to neuropathy due to inherited metabolic diseases is not well known but various factors can lead to it. They include diabetic polyneuropathy.

-Morphology: NA.

-Histology: NA.

How does Neuropathy due to Inherited Metabolic Diseases Present? 

Patients with neuropathy due to inherited metabolic disorders typically are males and females present at the age range of 60-70 years. The symptoms, features, and clinical findings associated with neuropathy due to inherited metabolic diseases include numbness, difficulty in walking, lethargy, poor appetite, 

How is Neuropathy due to Inherited Metabolic Diseases Diagnosed? 

Neuropathy due to inherited metabolic diseases is diagnosed through history taking, physical examination, sensory and motor testing, urinalysis, thyroid function tests.  

How is Neuropathy due to Inherited Metabolic Disorders Treated? 

Neuropathy due to inherited metabolic diseases is treated and management of the underlying metabolic disorder is the main approach. 

What is the Prognosis of Neuropathy due to Inherited Metabolic Diseases?

The prognosis of neuropathy due to inherited metabolic diseases is good depending on how well you will manage the underlying condition.

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Last updated on May 23, 2022