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[Multicystic encephalomalacia in an adult--a case report]
Insights
Multicystic encephalomalacia (MCE) in adults, resulting from infant anoxic-ischemic events, can present with minimal neurological deficits despite severe brain morphology changes. This case highlights brain plasticity
Area of Science:
- Neurology
- Neuroscience
- Pediatric Neurology
Background:
- Multicystic encephalomalacia (MCE) is typically associated with severe psychomotor retardation due to perinatal hypoxia or other insults to the immature brain.
- The condition often results from various damaging factors affecting early infancy brain development, leading to significant neurodevelopmental impairments.
Observation:
- A 35-year-old male presented with unsteadiness, moderate intellectual disability, and right-hand clumsiness following a shock state from a mismatched blood transfusion at 15 months.
- Neuroimaging (CT and MRI) revealed extensive bilateral cerebral white matter cystic lesions, sparing basal ganglia, cerebellum, and brainstem.
- Neurological examination showed only hyperreflexia, with no metabolic defects identified.
Findings:
- The diagnosis of MCE was established based on an anoxic-ischemic episode in infancy, a static clinical presentation, and characteristic cystic lesion distribution.
- A notable discrepancy exists between the severe morphological brain changes and the relatively mild neurological deficits observed in the patient.
- The patient's outcome suggests that significant brain plasticity may persist even after major insults, potentially mitigating functional impairment.
Implications:
- This case suggests that the timing of brain injury, particularly after 15 months of age when myelination is advanced, may influence the degree of neurological deficit.
- Preserved brain plasticity in later infancy could allow for functional compensation, leading to less severe outcomes than typically expected with extensive MCE.
- Further research into the neuroplasticity mechanisms in cases of early brain injury is warranted to understand the variability in clinical outcomes.
Abstract:
A case of multicystic encephalomalacia found in adult life was described. A 35-year-old man was admitted to our hospital with a chief complaint of unsteadiness. He had developed normally until he fell into the shock state induced by mismatch blood transfusion at the age of 15 months. Since then he has been mentally retarded moderately and had clumsiness of the skillful movement in the right hand. The other neurological abnormality was hyperreflexia only. Laboratory examination failed to disclose metabolic defect. Both CT scan and MRI demonstrated numerous cystic lesions of various size spreading over bilateral cerebral white matter partially involving the inner layer of the cortex. On the contrary basal ganglia, cerebellum and brainstem were completely spared. The diagnosis of MCE was made from (1) anoxic-ischemic episode in infancy, (2) static clinical picture and (3) characteristic distribution of cystic lesions. It is well known that MCE results from perinatal hypoxia, but it is a polyetiologic condition caused by various damages to immature brain of early infancy and usually results in severe psychomotor retardation. Nonetheless, it is intriguing in our case that marked discrepancy was found between morphological change and neurological deficit. It is probable that at the age of 15 months the myelination of major projecting fibers was almost completed, but sufficient plasticity was preserved in immature brain. As a result, the patient had the neurological deficit in the minimum degree in spite of severe morphological change.