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Hyperosmolar hyperglycemic state in advanced amyotrophic lateral sclerosis
Toshio Shimizu1, Masayuki Honda, Takuya Ohashi
1Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan. toshio_shimizu@tmhp.jp
Summary
Advanced amyotrophic lateral sclerosis (ALS) patients can develop hyperosmolar hyperglycemic state (HHS) due to severe muscle loss. Prompt treatment with hydration and insulin resolves HHS without chronic medication needs.
Area of Science:
- Neurology
- Endocrinology
- Metabolic Disorders
Background:
- Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease affecting motor neurons.
- Hyperosmolar hyperglycemic state (HHS) is a serious complication of diabetes characterized by extreme hyperglycemia and dehydration.
- The relationship between ALS and HHS is not well-established.
Observation:
- Five ventilator-dependent ALS patients without prior diabetes developed severe hyperglycemia (755-1544 mg/dl) following infections.
- Patients exhibited significant muscle wasting and tetraplegia.
- Initial HHS symptoms included fever, drowsiness, and polyuria.
Findings:
- Hydration and intravenous insulin therapy effectively resolved HHS in all patients, negating the need for chronic glucose-lowering medication.
- Oral glucose tolerance tests revealed insulin resistance and impaired early-phase insulin secretion, with preserved total insulin secretion.
- Severe skeletal muscle loss, a primary glucose consumer, combined with impaired early insulin release, is implicated as a cause of HHS in advanced ALS.
Implications:
- This study highlights a potential metabolic complication in advanced ALS patients.
- Understanding the pathophysiology of HHS in ALS can guide clinical management and prompt intervention.
- Further research into muscle-related metabolic dysregulation in neurodegenerative diseases is warranted.
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