Central pontine myelinolysis
Central pontine myelinolysis
Classification & external resources
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| Pons labeled at bottom left |
| ICD-10 |
G37.2 |
| DiseasesDB |
2198 |
| MedlinePlus |
000775 |
| eMedicine |
neuro/50 |
| MeSH |
D017590 |
Central pontine myelinolysis is a neurologic disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons.
Causes
The most common cause is the rapid correction of low blood sodium levels (hyponatremia).
Some scholars postulate that the real cause might be the lack of a substance that is essential for brain activity and is lacking due to malnutrition.
The fact that this condition is most frequently observed in patients with general ill health (alcoholism, cachexia etc.) is in accordance with this assumption. [1]
Diagnosis
Imaging by MRI demonstrates an area of high signal return on T2 weighted images.
Symptoms
Frequently observed symptoms in this disorder are sudden para or quadraparesis, dysphagia, dysarthria, double vision and loss of consciousness. The patient may experience locked-in syndrome where cognitive function is intact, but all muscles are paralyzed with the exception of eye blinking.
Treatment
To avoid myelinolysis, the correction of hyponatremia should not exceed 1 mEq/L per hour. [2][3]
References
- ^ Adams RA, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholics and malnourished patients. Arch Neurol Psychiatry. 1959;81:154–72. PMID 13616772
- ^ Kleinschmidt-DeMasters BK, Norenberg MD. Rapid correction of hyponatremia causes demyelination: relation to central pontine myelinolysis. Science. 1981;211(4486):1068-70. PMID 7466381
- ^ Laureno R. Experimental pontine and extrapontine myelinolysis. Trans Am Neurol Assoc. 1980;105:354-8. PMID 7348981
- Gocht A , Colmant HJ. Central pontine and extrapontine myelinolysis: a report of 58 cases. Clin Neuropath. 1987;6:262–70. PMID 3322623
- Menger H , Jorg J. Outcome of central pontine and extrapontine myelinolysis (n=44). J Neurol. 1999;246:700–5. PMID 10460448
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Nervous system pathology, primarily CNS (G00-G47, 320-349) |
Inflammatory diseases
of the CNS |
Meningitis (Arachnoiditis) - Encephalitis - Myelitis - Encephalomyelitis (Acute disseminated) - Tropical spastic paraparesis |
Systemic atrophies
primarily affecting the CNS |
Huntington's disease - Spinocerebellar ataxia (Friedreich's ataxia, Ataxia telangiectasia, Hereditary spastic paraplegia)
Spinal muscular atrophy: Werdnig-Hoffman disease - Kugelberg-Welander disease - Fazio Londe syndrome -
MND (Amyotrophic lateral sclerosis (ALS), Progressive muscular atrophy (PMA), Progressive bulbar, Pseudobulbar, PLS) |
Extrapyramidal and
movement disorders |
Parkinson's disease - Neuroleptic malignant syndrome - Postencephalitic parkinsonism - Pantothenate kinase-associated neurodegeneration - Progressive supranuclear palsy - Striatonigral degeneration - Dystonia (Spasmodic torticollis, Meige's syndrome, Blepharospasm) - Essential tremor - Myoclonus - Chorea (Choreoathetosis) - Restless legs syndrome - Stiff person syndrome |
Other degenerative /
demyelinating diseases |
Alzheimer's disease - Pick's disease - Alpers' disease - Dementia with Lewy bodies - Leigh's disease - Multiple sclerosis - Devic's disease - Central pontine myelinolysis - Transverse myelitis |
| Seizure/epilepsy |
Focal (Simple partial, Complex partial) - Generalised (Tonic-clonic, Absence, Atonic, Benign familial neonatal) - Lennox-Gastaut - West - Epilepsia partialis continua - Status epilepticus (Complex partial status epilepticus) |
| Headache |
Migraine (Familial hemiplegic) - Cluster - Vascular - Tension |
| Vascular |
Transient ischemic attack (Amaurosis fugax, Transient global amnesia) - Cerebrovascular disease (MCA, ACA, PCA, Foville's syndrome, Millard-Gubler syndrome, Lateral medullary syndrome, Weber's syndrome, Lacunar stroke) |
| Sleep disorders |
Insomnia - Hypersomnia - Sleep apnea (Ondine's curse) - Narcolepsy - Cataplexy - Kleine-Levin syndrome - Circadian rhythm sleep disorder - Delayed sleep phase syndrome - Advanced sleep phase syndrome |
| Other |
Hydrocephalus (Normal pressure) - Idiopathic intracranial hypertension - Encephalopathy - Brain herniation - Cerebral edema - Reye's syndrome - Syringomyelia - Syringobulbia - Spinal cord compression |
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