Demyelinating disease

Demyelinating disease
Classification and external resources
Photomicrograph of a demyelinating MS-Lesion. Immunohistochemical staining for CD68 highlights numerous macrophages (brown). Original magnification 10×.
ICD-10	G 35-G 37, G 61.0
ICD-9	340-341, 357.0
MeSH	D003711

A demyelinating disease is any disease of the nervous system in which the myelin sheath of neurons is damaged.^[1] This impairs the conduction of signals in the affected nerves, causing impairment in sensation, movement, cognition, or other functions depending on which nerves are involved.

The term describes the effect of the disease, rather than its cause; some demyelinating diseases are caused by genetics, some by infectious agents, some by autoimmune reactions, and some by unknown factors. Organophosphates, a class of chemicals which are the active ingredients in commercial insecticides such as sheep dip, weed-killers, and flea treatment preparations for pets, etc., will also demyelinate nerves.

Neuroleptics can cause demyelination.^[2]

Demyelinating diseases

Demyelinating diseases of the central nervous system include:

multiple sclerosis (together with the similar diseases called idiopathic inflammatory demyelinating diseases)
Vitamin B12 deficiency
Central pontine myelinolysis
Tabes Dorsalis
transverse myelitis
Devic's disease
progressive multifocal leukoencephalopathy
Optic neuritis
Leukodystrophies

Demyelinating diseases of the peripheral nervous system include:

Guillain-Barré syndrome and its chronic counterpart, chronic inflammatory demyelinating polyneuropathy
anti-MAG peripheral neuropathy
Charcot-Marie-Tooth Disease
Copper deficiency

References

^ "demyelinating disease" at Dorland's Medical Dictionary
^ Konopaske GT, Dorph-Petersen KA, Sweet RA, et al. (April 2008). "Effect of chronic antipsychotic exposure on astrocyte and oligodendrocyte numbers in macaque monkeys". Biol. Psychiatry 63 (8): 759–65. doi:10.1016/j.biopsych.2007.08.018. PMC 2386415. PMID 17945195. http://linkinghub.elsevier.com/retrieve/pii/S0006-3223(07)00847-5.

Pathology of the nervous system, primarily CNS (G04–G47, 323–349)

Inflammation

Brain	Encephalitis (Viral encephalitis, Herpesviral encephalitis) · Cavernous sinus thrombosis · Brain abscess (Amoebic)

Spinal cord	Myelitis: Poliomyelitis · Demyelinating disease (Transverse myelitis) · Tropical spastic paraparesis · Epidural abscess

Both/either	Encephalomyelitis (Acute disseminated) Meningoencephalitis

Brain/
encephalopathy

Degenerative

Extrapyramidal and movement disorders	Basal ganglia disease: Parkinsonism (PD, Postencephalitic, NMS) · PKAN · Tauopathy (PSP) · Striatonigral degeneration · Hemiballismus · HD · OA Dyskinesia: Dystonia (Status dystonicus, Spasmodic torticollis, Meige's, Blepharospasm) · Chorea (Choreoathetosis) · Myoclonus (Myoclonic epilepsy) · Akathesia Tremor (Essential tremor, Intention tremor) · Restless legs · Stiff person

Dementia	Tauopathy: Alzheimer's (Early-onset) · Frontotemporal dementia/Frontotemporal lobar degeneration (Pick's, Dementia with Lewy bodies) Multi-infarct dementia

Mitochondrial disease	Leigh's

Demyelinating

autoimmune (Multiple sclerosis, Neuromyelitis optica, Schilder's disease) · hereditary (Adrenoleukodystrophy, Alexander, Canavan, Krabbe, ML, PMD, VWM, MFC, CAMFAK syndrome) · Central pontine myelinolysis · Marchiafava-Bignami disease · Alpers' disease

Episodic/
paroxysmal

Seizure/epilepsy	Focal · Generalised · Status epilepticus · Myoclonic epilepsy

Headache	Migraine (Familial hemiplegic) · Cluster · Tension

Cerebrovascular	TIA (Amaurosis fugax, Transient global amnesia) Stroke (MCA, ACA, PCA, Foville's, Millard-Gubler, Lateral medullary, Weber's, Lacunar stroke)

Sleep disorders	Insomnia · Hypersomnia · Sleep apnea (Obstructive, Ondine's curse) · Narcolepsy · Cataplexy · Kleine-Levin · Circadian rhythm sleep disorder (Advanced sleep phase syndrome, Delayed sleep phase syndrome, Non-24-hour sleep-wake syndrome, Jet lag)

CSF

Intracranial hypertension (Hydrocephalus/NPH, Idiopathic intracranial hypertension) · Cerebral edema · Intracranial hypotension

Other

Brain herniation · Reye's · Hepatic encephalopathy · Toxic encephalopathy

Spinal cord/
myelopathy

Syringomyelia · Syringobulbia · Morvan's syndrome · Vascular myelopathy (Foix-Alajouanine syndrome) · Spinal cord compression

Both/either

Degenerative

SA	Friedreich's ataxia · Ataxia telangiectasia

MND	UMN only: PLS · PP · HSP LMN only: PMA · PBP (Fazio-Londe, Infantile progressive bulbar palsy) · Spinal muscular atrophies (SMA, SMARD1, SBMA, SMAX2, SMA-PCH) both: ALS

M: CNS

anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp

noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr

proc, drug(N1A/2AB/C/3/4/7A/B/C/D)

Multiple sclerosis and other demyelinating diseases of CNS (G35–G37, 340–341)

Signs and symptoms

Ataxia · Depression · Diplopia · Dysarthria · Dysphagia · Fatigue · Incontinence · Neurological fatigue · Nystagmus · Optic neuritis · Pain · Uhthoff's phenomenon · Dawson's fingers

Diagnosis and evolution following

McDonald criteria · Poser criteria · EDSS · Clinically isolated syndrome

Investigation

Pathophysiology · Experimental autoimmune encephalomyelitis

Treatment

Interferon beta-1a · Interferon beta-1b · Glatiramer acetate · Mitoxantrone · Natalizumab · Fingolimod · Therapies under investigation

Borderline forms

Acute disseminated encephalomyelitis · Balo concentric sclerosis · Neuromyelitis optica · Marburg multiple sclerosis · Schilder's disease · Tumefactive multiple sclerosis

(Guillain-Barré syndrome and CIDP are PNS)

Other

List of people with multiple sclerosis · List of multiple sclerosis organizations

M: CNS

anat(n/s/m/p/4/e/b/d/c/a/f/l/g)/phys/devp

noco(m/d/e/h/v/s)/cong/tumr, sysi/epon, injr

proc, drug(N1A/2AB/C/3/4/7A/B/C/D)

Nervous system pathology, PNS, somatic (G50–G64, 350–357)

Nerve, nerve root, plexus

Cranial nerve
disease

V (Trigeminal neuralgia, Anesthesia dolorosa) · VII (Facial nerve paralysis, Bell's palsy, Melkersson–Rosenthal syndrome, Parry–Romberg syndrome, Central seven) · XI (Accessory nerve disorder)

Radiculopathy,
plexopathy

brachial plexus (Brachial plexus lesion, Thoracic outlet syndrome) · Phantom limb

Mono-
neuropathy

Upper limb	median nerve (Carpal tunnel syndrome, Ape hand deformity) ulnar nerve (Ulnar nerve entrapment, Froment's sign, Guyon's canal syndrome, Ulnar claw) radial nerve (Radial neuropathy, Wrist drop, Cheiralgia paresthetica) long thoracic nerve (Winged scapula)

Lower limb	lateral cutaneous nerve of thigh (Meralgia paraesthetica) tibial nerve (Tarsal tunnel syndrome) plantar nerve (Morton's neuroma) superior gluteal nerve (Trendelenburg's sign) sciatic nerve (Piriformis syndrome)

General	Causalgia · Mononeuritis multiplex · Neuropathy (Neuralgia/Neuritis) · Nerve compression syndrome

Polyneuropathies/Polyradiculoneuropathy

HMSN	Charcot–Marie–Tooth disease · Dejerine–Sottas disease · Refsum's disease · Hereditary spastic paraplegia

Autoimmune/demyelinating	Guillain–Barré syndrome · Chronic inflammatory demyelinating polyneuropathy

Other	Alcoholic polyneuropathy

M: PNS

anat(h/r/t/c/b/l/s/a)/phys(r)/devp/prot/nttr/nttm/ntrp

noco/auto/cong/tumr, sysi/epon, injr

proc, drug(N1B)

Demyelinating disease

Demyelinating diseases

See also

References