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Demyelinating diseases are traditionally classified into two types: demyelinating myelinoclastic diseases and demyelinating leukodystrophic diseases. In the first group, a healthy and normal myelin is destroyed by toxic substances, chemicals, or autoimmune reactions.
Demyelinating diseases of the CNS can be classified according to their pathogenesis into five non-exclusing categories: demyelination due to inflammatory processes, viral demyelination, demyelination caused by acquired metabolic derangements, hypoxic–ischaemic forms of demyelination and demyelination caused by focal compression. [3]
CD68 immunostaining demonstrating macrophages and giant cells in a case of xanthogranulomatous pyelonephritis CD68 ( C luster of D ifferentiation 68) is a protein highly expressed by cells in the monocyte lineage (e.g., monocytic phagocytes , osteoclasts ), by circulating macrophages , and by tissue macrophages (e.g., Kupffer cells , microglia ).
Inflammatory demyelinating diseases (IDDs), sometimes called Idiopathic (IIDDs) due to the unknown etiology of some of them, are a heterogenous group of demyelinating diseases - conditions that cause damage to myelin, the protective sheath of nerve fibers - that occur against the background of an acute or chronic inflammatory process.
The name comes from a traditional classification of demyelinating diseases in two groups: demyelinating myelinoclastic diseases and demyelinating leukodystrophic diseases. In the first group, a normal and healthy myelin is destroyed by a toxic, chemical, or autoimmune substance. In the second group, myelin is abnormal and degenerates. [17]
Central pontine myelinolysis; Other names: Osmotic demyelination syndrome, central pontine demyelination: Axial fat-saturated T2-weighted image showing hyperintensity in the pons with sparing of the peripheral fibers, the patient was an alcoholic admitted with a serum Na of 101 treated with hypertonic saline, he was left with quadriparesis, dysarthria, and altered mental status
Specific types of leukodystrophy include the following with their respective ICD-10 codes when available: [citation needed] (E75.2) Alexander disease (E75.2) Canavan disease (E75.2) Hypomyelinating leukodystrophy type 7 (4H syndrome) (E75.2) Krabbe disease (E75.2) Metachromatic leukodystrophy (E75.2) Pelizaeus–Merzbacher disease
The disorder is sometimes called chronic relapsing polyneuropathy (CRP) or chronic inflammatory demyelinating polyradiculoneuropathy (because it involves the nerve roots). [2] CIDP is closely related to Guillain–Barré syndrome and it is considered the chronic counterpart of that acute disease. [ 3 ]