Lissencephaly in an adult Australian Kelpie

Abstract

This is the first report of lissencephaly in the Australian Kelpie and would suggest that some dogs with the condition can be managed with long-term anticonvulsant medication.

Lissencephaly in a Pekingese

“…The clinical findings of the present case, such as the early onset of seizures, absence of the menace responses, behavioral abnormalities, and difficulty in house- training were consistent with those in previously reported cases [ 7 , 10 , 14 , 19 , 21 ]. Quadrigeminal cisternal cysts can also be the cause of early onset of seizures [ 5 , 15 , 20 ].…”

“…Classical lissencephaly is distinguished from cobblestone cortical malformation by the abnormally thick cortex and absence of other brain malformations, like microcephaly, agenesis of the corpus callosum, or cerebellar hypoplasia [ 8 ]. Based on the MRI findings, the present and previously described cases [ 7 , 10 , 14 , 19 , 21 ] are equivalent to classical lissencephaly in humans. Classical lissencephaly is further divided, based on imaging results, into 6 grades, from severe grade 1 (comlete agyria) to mild grade 6 (subcortical band heterotopia only) [ 8 ].…”

“…In humans, lissencephaly has been found to be linked to mutations in several genes that have roles in neuronal migration [ 3 , 4 , 12 ]. In veterinary medicine, lissencephaly is an uncommon condition, with only a small number of cases reported in the literature as an isolated disorder [ 7 , 10 , 14 , 19 , 21 ]. Among those cases, Lhasa Apso appears to be overrepresented [ 10 , 19 , 21 ].…”

“…The MRI results of the present case, showing diffuse agyria with a few shallow undulations over the frontal and temporal poles, were consistent with grade 2a. In humans, the severity of lissencephaly is thought to determine prognosis [ 2 ], however it is unclear whether the same is true for dogs given that only 1 case, classified as grade 2b, was previously applied to this grading system [ 7 ]. Nevertheless, dogs with lissencephaly appear to be well controlled by anticonvulsants and live a long life [ 7 ].…”

“…Until recently, reports of lissencephaly in dogs were based on postmortem findings [ 10 , 21 ]. On the other hand, because MRI has become more readily available in veterinary medicine, antemortem diagnoses of lissencephaly have been made by imaging findings alone [ 7 , 14 , 19 ]. In fact, the diagnosis of lissencephaly is usually made by ultrasound, computed tomography, or MRI in humans [ 2 , 13 , 17 ].…”