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[Ataxic cerebral palsy and brain imaging]
Insights
This study presents five cases of ataxic cerebral palsy, highlighting diverse neurological presentations and imaging findings. The cases underscore that ataxic cerebral palsy encompasses various conditions with initial hypotonia evolving into ataxic symptoms.
Area of Science:
- Neurology
- Pediatrics
- Radiology
Context:
- Ataxic cerebral palsy is a clinical diagnosis encompassing diverse etiologies.
- Early-onset hypotonia progressing to ataxic gait is a common presentation.
- Brain imaging plays a crucial role in differentiating subtypes.
Purpose:
- To present five distinct cases diagnosed with ataxic cerebral palsy.
- To correlate clinical symptoms with neuroimaging findings (MRI and CT).
- To illustrate the heterogeneity of conditions presenting as ataxic cerebral palsy.
Summary:
- Case 1: Early-onset inherited cerebellar ataxia with cerebellar and pontine atrophy.
- Case 2: Cerebellar atrophy in a child with early-onset ataxic gait.
- Case 3 & 4: Spino-ponto-cerebellar atrophy diagnosed as Marinesco-Sjörgren syndrome.
- Case 5: Cranium bifidum and medullar velum agenesis associated with ataxic symptoms.
Impact:
- Highlights the broad spectrum of neurological disorders classified under ataxic cerebral palsy.
- Emphasizes the importance of detailed neuroimaging for accurate diagnosis and subtyping.
- Contributes to understanding the varied etiologies and clinical trajectories of early-onset ataxia.
Abstract:
Five cases diagnosed as having ataxic cerebral palsy were presented with their brain imaging. Case 1, a 3-year-old-girl had been floppy since 7 months of age and began ataxic walk with spastic legs from 18 months of age. MRI revealed generalized atrophy of cerebellum (especially in anterior superior part) and slight atrophy of pons. Her mother also had ataxia with spastic legs of early onset. She and her mother were thought to have an early-onset inherited non-progressive cerebellar ataxia syndrome. Case 2, a 8-year-old-girl had ataxic walk since 17 months of age. MRI revealed cerebellar atrophy especially in anterior superior part. Case 3, a 10-year-old boy was floppy since 4 months of age and suspected as ataxic at 4 years of age. He could walk only with cruches. He had dwarfism and cataracts since 4 years of age. CT and MRI revealed generalized spino-ponto-cerebellar atrophy. Final diagnosis was Marinesco-Sjörgren syndrome. Case 4, a 10-year-old girl had opisthotonus and floppiness since 4 months of age. She could walk only with cruches. CT and MRI revealed generalized spino-ponto-cerebellar atrophy. Case 5, a 8-year-old boy showed head nodding and nystagmus since 4 months of age. He started ataxic gait at 8 years of age. He could vocalize only single sound for speech. MRI revealed cranium bifida and agenesis of anterior medullar velum. Ataxic cerebral palsy is the term often used to describe very different conditions, the clinical picture starts as hypotonia and changes into the ataxic symptoms in a few years.(ABSTRACT TRUNCATED AT 250 WORDS)