The osmotic demyelination syndrome includes pontine and extrapontine myelinolysis, the first being its classic form of presentation. Usually related to rapid corrections of hyponatremia (serum sodium less than 135mEq/L), it has been described in multiple conditions that can alter plasma osmolality. The symptoms are varied and include spastic tetra paresis, pseudobulbar paralysis, coma, seizures or the characteristic “locked in” syndrome, as well as behavioral disorders such as lack of impulse control, aggressiveness, depressive syndromes, catatonia, mutism, and emotional lability. Years ago, it was a very difficult entity to diagnose, but with the introduction of magnetic resonance imaging, many oligosymptomatic or asymptomatic cases have been detected, although there is no known treatment, spontaneous recovery can occur.
Hiponatremia
Neuroimagen en síndrome de desmielinización osmótica. Neuroimaging in asmotic demyelination syndrome
Demencia por Cuerpos de Lewy, un Reto Diagnóstico. Dementia With Lewy Bodies, A Diagnostic Challenge.
Dementia with Lewy bodies is a neurodegenerative disease of unknown etiology, it is the second cause of dementia of the sixth decade of life; Its diagnosis is a challenge, because certain signs and symptoms that it presents are typical of Parkinson’s Disease and Alzheimer’s Disease. The following case report is one of the few documented patients with Dementia with Lewy bodies in Ecuador. We report this in order to state the diagnostic difficulty that this pathology generates and describe the main characteristics that differentiate it from other dementia syndromes, highlighted in the recently updated criteria by the Consortium of Dementia with Lewy bodies. A meticulous neurological examination and neuropsychological assessment were essential in the study and prognosis of the patient. Dementia with Lewy bodies requires a thorough diagnosis, due to the challenge that originates its early recognition; the criteria described accelerated their recognition due the update of the recommendations on the clinical diagnosis of Dementia with Lewy bodies.
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Parkinsonismo y Mielinolisis Osmótica. Descripción de caso
Introduction: Osmotic demyelination syndrome is a rare medical condition in which parkinsonian signs are relatively non frequent. Objective: To report a case of a patient with hyponatremia rapidly corrected and treated with neuroleptics. The cranial MRI showed demyelinating lesions in pons and basal ganglia. The patient clinically developed parkinsonism, with no response to dopamine. Conclusion: The inadequate correction of hyponatremia causes demyelinating lesions. As we appreciate in this case, the prescription of neuroleptic drugs may negatively affect the response to dopaminergic therapy.
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Mielinólisis Extrapontina Secundaria a Hiponatremia por Crisis Adrenal
Introduction: Extrapontine myelinolysis is an acute unusual process, included in demyelinating syndromes of metabolic etiology, which differentiate from central pontine myelinolysis, by affecting other brain areas with clinical manifestation such as dystonia, catatonia, postural tremor, myoclonic spasm, parkinsonism or pyramidal sings.
Objective: Present clinical, electrolytic, hormonal and neuroradiologic findings with emphasis in the unusual location of this disease.
Conclusion: Unlike central pontine myelinolysis, extrapontine has a lower incidence. Trismus, manifested in this patient is an unusual sign. The treatment is only supportive, with a high probability of mortality.
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Sindrome parkinsoniano “rígido-acinético” debido a mielinosis extrapontina y mielinosis pontina asintomática secundarios a corrección rápida de hiponatremia.
The osmotic demyelinating syndromes are not always restricted to the pons. The extrapontine myelinolysis is seen in 10% of patients with central pontine myelinolysis. We present a 62-year-old woman who developed hyponatremia due to repeated vomiting. An acute akinetic-rigid syndrome followed rapid correction of hyponatremia. Bilateral putaminal and caudate lesions were seen on MRI. There were no clinical features of pontine involvement even though a typical lesion was seen on MRI. She recovered well with only symptomatic treatment. Although the exact pathogenesis is unknown, the most widely accepted hypothesis is that cellular edema secondary to the fluctuating osmotic forces, with rapid correction of hyponatremia, results in fibre-tract compression and demyelination. In contrast to the generally expected poor outcome in this condition, patients do have good recovery with symptomatic treatment and good nursing care.
