Polymicrogyria Natalie Crawford Karen Inzana D. Phillip Sponenberg Virginia – Maryland Regional...
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Transcript of Polymicrogyria Natalie Crawford Karen Inzana D. Phillip Sponenberg Virginia – Maryland Regional...
Polymicrogyria
Natalie CrawfordKaren Inzana
D. Phillip Sponenberg
Virginia – Maryland Regional College of Veterinary MedicineBlacksburg, Virginia
Presented at SEVPAC 2008 – Permission granted for use
on SEVPAC website only
Polymicrogyria (also known as microgyria)
Malformation of the cerebral cortex Neuronal migration defect leading to
abnormal lamination Excessive production of gyri Associated with other brain malformations
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
website only
Veterinary Medicine
Ruminants Cattle
Sheep
Rats Experimentally induced
DogsPresented at SEVPAC 2008 –
Permission granted for use on SEVPAC website only
Historical Perspective
Van Winkle et. al. (1994) Four related Standard Poodle dogs with cortical blindness Young dogs (5-9 months)
Jurney et. al. (2008) Four un-related Standard Poodle dogs with cortical
blindness and behavioral abnormalities Three young dogs (3-4 months) One older dog (5 years)
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
website only
Allegro
3 month old male intact Standard Poodle
Decreased vision Noticed from 9 weeks of age
Abnormal behavior
Possible decreased hearing
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website only
Diagnosis
Ophthalmic Examination No ophthalmic cause for blindness
Neurologic Examination Generalized or diffuse encephalopathy Cortical blindness
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website only
Other Findings
Gross lesions - Definitive
Histologic lesions
Imaging (MRI) Not done in Allegro’s case
Brain Activity Monitoring (EEG) Not done in Allegro’s case
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
website only
Gross Findings
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Histologic Lesions
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Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
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Normal Nissl
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H&E (07-1996.4)
Polymicrogyria H & E
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Polymicrogyria Nissl
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
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Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
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Polymicrogyria Phenotype
Clinical Presentation Standard Poodles 0-6 months of age Cortical Blindness Gross lesions
Outcome Euthanasia
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Human Medicine
Frequent occurrence
Manifests with varying degrees of mental retardation, developmental delays and seizure activity
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Schulyer Hudson
Bilateral Persylvian Polymicrogyria Partial paralysis of
muscles of the face, jaws and throat
Learning difficulties Cerebral palsy Seizures
Genetic
www.darn-tootin.comPresented at SEVPAC 2008 – Permission granted for use on SEVPAC
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Literature
Veterinary Neuropathology, Summers B, et al. (1995)
Blindness Due to Polymicrogyria and Asymmetrical Dilation of the Lateral Ventricles in Standard Poodles, Van Winkle TJ, et al. (1994)
Neuropathology: A Reference Text of CNS Pathology, 2nd Ed., Ellison D, et al. (2004)
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
website only
Acknowledgements
D. Phillip Sponenberg Karen Inzana Carrie Jurney
Jurney C, et al. Polymicrogyria in Four Standard Poodles: A Case Series with Histopathologic, Electroencephalographic
and Magnetic Resonance Imaging Findings.
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
website only
Contributors
Jamie Haddad – UPenn Thomas Van Winkle – UPenn Andrew Miller – Cornell Andras Komaromy – UPenn Charles Vite - UPenn Gayle Johnson - Missouri Dennis O’Brien – Missouri
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
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Questions?
Presented at SEVPAC 2008 – Permission granted for use on SEVPAC
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