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Abstract

Objective: To define the behavioral profile of periventricular nodular heterotopia (PNH), a malformation of cortical development that is associated with seizures but reportedly normal intelligence, and to correlate the results with anatomic and clinical features of this disorder.
Methods: Ten consecutive subjects with PNH, all with epilepsy and at least two periventricular nodules, were studied with structural MRI and neuropsychological testing. Behavioral results were statistically analyzed for correlation with other features of PNH.
Results: Eight of 10 subjects had deficits in reading skills despite normal intelligence. Processing speed and executive function were also impaired in some subjects. More marked reading difficulties were seen in subjects with more widely distributed heterotopia. There was no correlation between reading skills and epilepsy severity or antiepileptic medication use.
Conclusion: The neuronal migration disorder of periventricular nodular heterotopia is associated with an impairment in reading skills despite the presence of normal intelligence.

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Letters to the Editor
14 June 2005
Reading impairment in the neuronal migration disorder of periventricular nodular heterotopia
Deborah K. Sokol, Indiana University School of Medicine
Meredith R. Golomb, Karen S. Carvahlo, Mary Edwards-Brown

We read with interest the report by Chang et al on the association of bilateral periventricular nodular heterotopia with seizures and impaired reading in people with normal intelligence.

We performed extensive neuropsychological testing on a 16-year-old boy with a left perventricular heterotopion and left temporal lobe dysplasia diagnosed by magnetic resonance imaging and did not find any evidence of reading disability. Our patient came to evaluation because of academic difficulties. At the time of testing he had focal epilepsy which was well-controlled on lamotrigine. On the Wechsler Intelligence Scale for Children-III Edition, he had a Full Scale intelligence quotient (IQ) of 104, a Verbal IQ of 114, and a Performance IQ of 93. His Processing Speed Index was 64 (1 %tile). On the Gray Oral Reading Test-4, his Reading Quotient was 103 (58th %tile for age) with adequate reading rate, accuracy, frequency, and comprehension for age. On the Luria Nebraska Neuropsychologic Battery- Form 1, none of the 11 clinical scales or 5 ancillary scales were above the critical level of sixty-one. On the Wide Range Achievement Test-Edition III, his reading standard score was 94 at the high school level (34 %tile).

Our patient had normal reading ability. His slow processing speed contributed to his academic difficulty. This case supports the trend described by Chang et al that patients with fewer heterotopia show less cognitive impairment. We hypothesize that children with unilateral periventricular heterotopia may be less likely to develop reading impairment than children with bilateral heterotopia because there is less cortical involvement.

14 June 2005
Reply from authors
Bernard S. Chang, Beth Israel Deaconess Medical Center and Harvard Medical School

We appreciate the interest of Sokol et al in our findings. Their results support our demonstration of a relationship between the anatomical distribution of heterotopia and the cognitive consequences. Our study did not include any subjects with only a single heterotopic nodule or with other coexistent malformations, as in her case. We believe there are many potential mechanisms by which the presence of gray matter heterotopia could lead to a specific cognitive deficit, and further work is required to clarify them.

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Published In

Neurology®
Volume 64Number 5March 8, 2005
Pages: 799-803
PubMed: 15753412

Publication History

Published online: March 7, 2005
Published in print: March 8, 2005

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Authors

Affiliations & Disclosures

B. S. Chang, MD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
J. Ly, BA
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
B. Appignani, MD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
A. Bodell, MS
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
K. A. Apse, ScM
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
R. S. Ravenscroft, BA
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
V. L. Sheen, MD, PhD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
M. J. Doherty, MD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
D. B. Hackney, MD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
M. O’Connor, PhD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
A. M. Galaburda, MD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.
C. A. Walsh, MD, PhD
From the Division of Neurogenetics (Drs. Chang, Sheen, and Walsh, A. Bodell, K.A. Apse, and R.S. Ravenscroft), Behavioral Neurology Unit (Drs. O’Connor and Galaburda, J. Ly), and Howard Hughes Medical Institute (Dr. Walsh, A. Bodell), Department of Neurology, and Division of Neuroradiology (Drs. Appignani and Hackney), Department of Radiology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, and Swedish Neuroscience Institute (Dr. Doherty), Epilepsy Center, Seattle, WA.

Notes

Address correspondence and reprint requests to Dr. B.S. Chang, Department of Neurology, Beth Israel Deaconess Medical Center, NRB 02-268B, 77 Ave. Louis Pasteur, Boston, MA 02115; e-mail: [email protected]

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