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Primary lateral sclerosis in a child
M L Grunnet1, C Leicher, A Zimmerman
1Department of Neurology, University of Connecticut Health Center, Farmington 06032.
Insights
Primary lateral sclerosis (PLS) is a rare neurological disorder. This case study details a child with typical PLS clinical and pathological findings, previously undescribed in pediatric patients.
Area of Science:
- Neurology
- Pediatric Neurology
- Neurodegenerative Diseases
Background:
- Primary lateral sclerosis (PLS) is a rare, adult-onset neurodegenerative disorder.
- It is characterized by progressive spasticity and corticospinal tract degeneration.
- PLS has not been previously described in pediatric populations.
Observation:
- A case report of an infant presenting with progressive motor deterioration before 12 months of age.
- The infant exhibited generalized spasticity.
- The child expired at 47 months of age.
Findings:
- Neuropathologic examination revealed exclusive degeneration of the corticospinal tracts.
- The clinical and pathological findings were consistent with typical Primary lateral sclerosis.
- This represents the first documented case of PLS in a pediatric patient.
Implications:
- This case expands the known age spectrum for Primary lateral sclerosis.
- It highlights the importance of considering PLS in the differential diagnosis of pediatric spasticity.
- Further research is needed to understand the etiology and long-term outcomes of pediatric PLS.
Abstract:
Primary lateral sclerosis (PLS), previously undescribed in children, is characterized by a spastic motor deterioration and pathologic demonstration of corticospinal tract degeneration. We report an infant who, before 12 months of age, developed a progressive motor disease characterized by generalized spasticity. He died at 47 months, and neuropathologic examination revealed only corticospinal tract degeneration. We conclude that this child had typical clinical and pathologic findings for PLS.