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Acute cerebral symptomatology, a rare presentation of scleromyxedema
Abstract:
A 65 year old male with the entity, scleromyxedema, experienced exacerbation of the disease in which the main clinical features involved the central nervous system. He presented with clouded sensorium, disorganized thinking, combative behavior, headache, unsteady gait and grand mal seizures. A few days after hospital admission the symptoms abated. After a 6 day hiatus, the symptoms suddenly recurred, continuing for another week. The symptomatology again suddenly ceased with complete clearance of mental status. During the full-blown delirium, the electroencephalogram had demonstrated diffuse slowing while lumbar puncture, brain scan, E.M.I. scan and cerebral arteriogram failed to contribute to the understanding of the clinical presentation. Scleromyxedema rarely involves the central nervous system. This case illustrates a very unusual manifestation of scleromyxedema, prominent central nervous system involvement presenting as an acute organic brain syndrome. It is the only case which includes formal mental status examination, cerebrospinal fluid findings and electroencephalogram results.
Insights
Scleromyxedema rarely affects the central nervous system. This case details a unique presentation of scleromyxedema with acute organic brain syndrome, including detailed neurological and EEG findings.
Area of Science:
- Neurology
- Dermatology
- Rare Diseases
Background:
- Scleromyxedema is a rare connective tissue disease characterized by mucin deposition in the dermis.
- Central nervous system (CNS) involvement in scleromyxedema is exceptionally uncommon.
Observation:
- A 65-year-old male with scleromyxedema presented with acute neurological symptoms including clouded sensorium, disorganized thinking, and seizures.
- The patient experienced fluctuating episodes of delirium, with temporary abatement followed by recurrence.
- Diagnostic workup, including electroencephalogram (EEG), lumbar puncture, and neuroimaging, was performed during the acute phase.
Findings:
- The electroencephalogram (EEG) revealed diffuse slowing during the delirium.
- Cerebrospinal fluid (CSF) analysis and neuroimaging studies did not reveal specific abnormalities.
- This case represents a unique instance of prominent CNS involvement in scleromyxedema, manifesting as an acute organic brain syndrome.
Implications:
- This case highlights an unusual neurological manifestation of scleromyxedema.
- It underscores the importance of considering CNS involvement in scleromyxedema exacerbations, even when rare.
- The detailed documentation of mental status, CSF, and EEG findings provides valuable data for understanding this rare condition.