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Abstract
Objective To determine whether specific speech, language, and oromotor profiles are associated with different patterns of polymicrogyria, we assessed 52 patients with polymicrogyria using a battery of standardized tests and correlated findings with topography and severity of polymicrogyria.
Methods Patients were identified via clinical research databases and invited to participate, irrespective of cognitive and verbal language abilities. We conducted standardized assessments of speech, oromotor structure and function, language, and nonverbal IQ. Data were analyzed according to normative assessment data and descriptive statistics. We conducted a correlation analysis between topographic pattern and speech and language findings.
Results Fifty-two patients (33 male, 63%) were studied at an average age of 12.7 years (range 2.5–36 years). All patients had dysarthria, which ranged from mild impairment to anarthria. Developmental speech errors (articulation and phonology), oral motor structure and function deficits, and language disorder were frequent. A total of 23/29 (79%) had cognitive abilities in the low average to extremely low range. In the perisylvian polymicrogyria group (36/52), speech, everyday language, and oral motor impairments were more severe, compared to generalized (1 patient), frontal (3), polymicrogyria with periventricular nodular heterotopia (3), parasagittal parieto-occipital (1), mesial occipital (1), and other (7) patterns.
Conclusions Dysarthria is a core feature of polymicrogyria, often accompanied by receptive and expressive language impairments. These features are associated with all polymicrogyria distribution patterns and more severe in individuals with bilateral polymicrogyria, particularly in the perisylvian region.
Glossary
- CELF=
- Clinical Evaluation of Language Fundamentals;
- CFCS=
- Communication Function Classification System;
- CI=
- confidence interval;
- DEAP=
- Diagnostic Evaluation of Articulation and Phonology;
- EDACS=
- Eating and Drinking Ability Classification System;
- PCC=
- percentage consonants correct;
- PEG=
- percutaneous endoscopic gastrostomy;
- PNH=
- periventricular nodular heterotopia;
- PPVT-4=
- Peabody Picture Vocabulary Test, Fourth Edition;
- TROG-2=
- Test of Reception of Grammar, Second Edition;
- VSS=
- Viking Speech Scale
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
CME Course: NPub.org/cmelist
- Received June 8, 2020.
- Accepted in final form January 6, 2021.
- © 2021 American Academy of Neurology
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