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罗威纳犬的良性家族性幼年癫痫

Benign familial juvenile epilepsy in Lagotto Romagnolo dogs.

作者信息

Jokinen T S, Metsähonkala L, Bergamasco L, Viitmaa R, Syrjä P, Lohi H, Snellman M, Jeserevics J, Cizinauskas S

机构信息

Department of Clinical Veterinary Sciences, University of Helsinki, Finland.

出版信息

J Vet Intern Med. 2007 May-Jun;21(3):464-71. doi: 10.1892/0891-6640(2007)21[464:bfjeil]2.0.co;2.

DOI:10.1892/0891-6640(2007)21[464:bfjeil]2.0.co;2
PMID:17552452
Abstract

BACKGROUND

Idiopathic childhood epilepsies with benign outcomes are well recognized in human medicine, but are not reported in veterinary literature. We recognized such a neurologic syndrome in Lagotto Romagnolo dogs.

ANIMALS

Twenty-five Lagotto Romagnolo puppies from 9 different litters examined because of simple or complex focal seizures and 3 adult Lagotto Romagnolo dogs exhibiting similar clinical signs were used.

METHODS

Clinical and diagnostic evaluations of affected dogs were conducted, including electromyography, electroencephalography, and other testing.

RESULTS

Seizures in puppies began at 5 to 9 weeks of age and usually resolved spontaneously by 8 to 13 weeks. Those with the most severe seizures also had signs of neurologic disease between these seizures, including generalized ataxia and hypermetria. There were no abnormalities in routine laboratory screenings of blood, urine, and cerebrospinal fluid. Electromyography, brainstem auditory-evoked potentials, and magnetic resonance imaging revealed no specific and consistent abnormalities. Fourteen of 16 (87.5%) affected puppies and 2 of 3 (67%) adult dogs revealed epileptiform activity in the electroencephalogram. Histopathologic examination in 1 puppy and 1 adult dog revealed lesions of Purkinje cell inclusions and vacuolation of their axons restricted to the cerebellum. Pedigree analysis suggests an autosomal recessive mode of inheritance.

CONCLUSIONS AND CLINICAL IMPORTANCE

This disorder, with simple or complex focal seizures and cerebellar lesions, represents a newly recognized epileptic syndrome in dogs.

摘要

背景

特发性儿童期癫痫伴良性转归在人类医学中已得到充分认识,但兽医文献中未见报道。我们在罗威纳拉戈托犬中发现了这样一种神经综合征。

动物

使用了来自9个不同窝的25只罗威纳拉戈托幼犬,这些幼犬因单纯性或复杂性局灶性癫痫发作接受检查,以及3只表现出类似临床症状的成年罗威纳拉戈托犬。

方法

对患病犬进行了临床和诊断评估,包括肌电图、脑电图及其他检查。

结果

幼犬癫痫发作始于5至9周龄,通常在8至13周龄时自发缓解。癫痫发作最严重的幼犬在发作间期也有神经疾病体征,包括全身性共济失调和辨距不良。血液、尿液和脑脊液的常规实验室检查未发现异常。肌电图、脑干听觉诱发电位和磁共振成像未显示特定且一致的异常。16只患病幼犬中有14只(87.5%)和3只成年犬中有2只(67%)脑电图显示癫痫样活动。对1只幼犬和1只成年犬的组织病理学检查发现,仅小脑存在浦肯野细胞包涵体病变及其轴突空泡化。系谱分析提示为常染色体隐性遗传模式。

结论及临床意义

这种伴有单纯性或复杂性局灶性癫痫发作及小脑病变的疾病代表了犬类一种新发现的癫痫综合征。

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