MRI Findings in Osmotic Myelinolysis

Chua, Gim Chuah; Sitoh, Yih Yian; Lim, C. C. Tchoyoson; Chua, H C; Ng, Puay Yong

doi:10.1053/crad.2002.0977

Cited by 55 publications

(27 citation statements)

References 12 publications

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“…Less commonly, lesions appear isointense relative to surrounding brain tissue on T1-weighted images. Lesions typically do not enhance after the administration of contrast material (2,13). Case reports have suggested that restricted diffusion may be seen earlier than the classic findings in areas of osmotic demyelination on T2-weighted images (5,14).…”

Section: Discussionmentioning

confidence: 99%

Osmotic Demyelination Syndrome

Howard¹,

Barletta²,

Klufas³

et al. 2009

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A 22-year-old woman with a history of drug abuse was found, by her mother, unconscious and unresponsive at a friend's house. It was reported that she had a 2-week history of unresponsiveness and vomiting. She was taken to a local hospital by emergency medical services. Upon admission, the patient experienced a generalized tonic-clonic seizure and was intubated for airway protection. She was markedly hyponatremic, her urine toxicology results were negative, and computed tomographic (CT) images of the head were reportedly normal (images unavailable). Her sodium level was rapidly corrected over the course of 11 hours. The initial sodium level was 113 mmol/L. Five hours later it was 124 mmol/L, and 6 hours after that it was 136 mmol/L. For the first few days of hospitalization, the patient remained completely unresponsive. On the 5th day of hospitalization, she began to spontaneously open and close her eyes, but she did not follow commands or track movements. Serial electroencephalograms showed a polymorphic delta rhythm, a finding consistent with severe hypoxic encephalopathy. Magnetic resonance (MR) imaging performed 2 weeks later reportedly showed extensive restricted diffusion throughout the cerebral cortex, thalami, and pons (images unavailable). Her mental status remained unchanged for approximately 2 months, at which time she was transferred to a tertiary care center. Upon admission to the tertiary care center, she was again noted to spontaneously open and close her eyes. Significant findings of neurologic examination were decerebrate posture, bilateral hypertonia in the upper limbs, Babinski reflex on the right side, and normal (downward) plantar reflex on the left.

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Section: Discussionmentioning

confidence: 99%

Osmotic Demyelination Syndrome

Howard¹,

Barletta²,

Klufas³

et al. 2009

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“…DWI has been reported to identify intramyelinic or cytotoxic edema, which may ensue from ischemia, infarction, infectious processes, tumors and demyelinating disorders [38, 39, 40]. Diffuse patterns of restricted diffusion can also be seen in the setting of metabolic derangements like hypo- and hyperglycemia, rapid correction of hyponatremia and in disorders of metabolism such as maple syrup urine disease, citrullinemia and methylmalonic acidemia [41, 42, 43, 44]. Diffusion defects associated with metabolic abnormalities may or may not be reversible depending on their etiology and the extent of brain tissue injury [41, 43, 44, 45].…”

Section: Discussionmentioning

confidence: 99%

“…Diffuse patterns of restricted diffusion can also be seen in the setting of metabolic derangements like hypo- and hyperglycemia, rapid correction of hyponatremia and in disorders of metabolism such as maple syrup urine disease, citrullinemia and methylmalonic acidemia [41, 42, 43, 44]. Diffusion defects associated with metabolic abnormalities may or may not be reversible depending on their etiology and the extent of brain tissue injury [41, 43, 44, 45]. In our patient with NTS, despite improvement in his nutritional status and normalization of his serum electrolytes and amino acids, the MRI abnormalities did not change.…”

Section: Discussionmentioning

confidence: 99%

“…In our patient with NTS, despite improvement in his nutritional status and normalization of his serum electrolytes and amino acids, the MRI abnormalities did not change. Additionally, MR spectroscopy revealed increased intraventricular lactate and choline, suggesting the possibility of demyelination, cellular breakdown or anaerobic metabolism [41, 43, 44, 45]. With diffuse volume loss and persistent diffusion defects at 17 months of age, some degree of permanent tissue injury is likely.…”

Section: Discussionmentioning

confidence: 99%

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Netherton Syndrome with Extensive Skin Peeling and Failure to Thrive due to a Homozygous Frameshift Mutation in <i>SPINK5</i>

et al. 2005

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Background: Netherton syndrome (NTS) is a rare autosomal recessive multisystem disorder characterized by congenital erythroderma and ichthyosis, hair shaft abnormalities and immune dysregulation. The disorder is caused by deleterious mutations in the SPINK5 gene, encoding the serine protease inhibitor LEKTI. Objective: Our objective was to investigate if the erythrodermic variant of peeling skin syndrome is also caused by SPINK5 mutations and to study the consequences of the disease on infantile brain development. Methods: In an infant with extensive erythroderma, peeling skin and failure to thrive, we analyzed the SPINK5 gene for pathogenic mutations by direct DNA sequencing and performed repeated brain MRI studies with diffusion-weighted imaging. Results: We identified a homozygous 4-base-pair insertion in exon 5 of SPINK5, which introduces a premature termination codon and appears to be a common mutation among West Indies islanders. MRI analyses revealed a persistent diffuse volume loss. Conclusion: Our results confirm that early truncation mutations of the coding sequence of SPINK5 produce a severe phenotype and that generalized peeling skin is one of the manifestations of NTS. We further demonstrate for the first time that NTS may be associated with MRI abnormalities indicative of a permanent tissue injury of the brain.

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“…CPM affects the pons and results in a symmetric bat-wing lesion. Imaging is often normal within the first two weeks of hyponatremia correction; thus, normal imaging does not exclude ODS [8,9,32]. We describe a case of fatal ODS that developed suddenly in a 13-year-old boy who previously had been successfully managed with corticosteroids, deamino arginine vasopressin (DDAVP) and thyroid hormone for postoperative panhypopituitarism for over a decade following the resection of a nasofrontal encephalocele in infancy.…”

Section: Introductionmentioning

confidence: 99%

Osmotic Myelinolysis with Malignant Cerebellar Edema Occurring after DDAVP-Induced Hyponatremia in a Child

Ranger¹,

Szymczak²,

Levin³

et al. 2010

Pediatr Neurosurg

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Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.

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MRI Findings in Osmotic Myelinolysis

Cited by 55 publications

References 12 publications

Osmotic Demyelination Syndrome

Osmotic Demyelination Syndrome

Netherton Syndrome with Extensive Skin Peeling and Failure to Thrive due to a Homozygous Frameshift Mutation in <i>SPINK5</i>

Osmotic Myelinolysis with Malignant Cerebellar Edema Occurring after DDAVP-Induced Hyponatremia in a Child

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