Hereditary polyneuropathy in the Alaskan Malamute.

OBJECTIVE: To prove the hypothesis that a polyneuropathy in Alaskan Malamutes has a genetic background. MATERIAL AND METHODS: Pedigrees of 131 related Alaskan Malamutes were included in the current study. Neurological examination, electrodiagnosis as well as muscle and nerve biopsies could be performed in 10 dogs. Information about the disease status of the other 121 Alaskan Malamutes were supplied by referring veterinarians, breeders and owners. Segregation analysis using four different models (monogenic, polygenic, mixed monogenic-polygenic and the phenotypic model) was performed on 71 dogs to test the different mechanisms of genetic transmission. RESULTS: In seven clinically affected dogs abnormal electromyographic findings and reduced nerve conduction velocity were detected. Suspected diagnosis of polyneuropathy was confirmed by nerve biopsy results, characterized by axonal degeneration and hypomyelination. Muscle specimens revealed signs of neurogenic myopathy. Three related clinically normal Alaskan Malamutes also displayed moderate neuromuscular changes in histopathology. In the segregation analysis the polygenic model proved as best suitable to explain the observed segregation pattern among all other models tested. CONCLUSION: The current study could demonstrate that polyneuropathy in Alaskan Malamutes is a hereditary disease with variable phenotypic expression ranging from severely affected to subclinical forms, which has to be considered in future gene analysis studies.

Intracranial amebic abscesses: CT and MR findings.

The CT and magnetic resonance findings in a case of intracranial amebic abscesses are described. Due to increased travel to endemic areas, amebic abscess should also be included in the differential diagnosis for patients who live in the temperate parts of the world.

[The multicentric glioblastoma multiforme]. / Das multizentrische Glioblastoma multiforme.

The authors report on two cases of multicentric glioblastoma multiforme, one of them being a trilocular glioblastoma, whereas in the second case a second focus developed contralaterally. Both patients received whole skull radiation of 40 Gy. The patient who suffered from the primarily unilocal tumour was given a tumour boost. The article reports on the imaging via CT and MR.

[CT in the preoperative clarification of Kartagener's syndrome]. / Die CT in der präoperativen Abklärung des kartagenen Syndroms.

A case of Kartagener's syndrome with situs inversus totalis that was seen on CT is presented. CT was employed to obtain important preoperative information.

[Tuberous sclerosis: family manifestations]. / Tuberöse Sklerose: familiärer Befall.

The authors report on 6 sisters and brothers with Bourneville's disease (tuberous sclerosis). The article describes the different morphologic manifestations of the illness in the patients and places them in relation with the generally assumed principal signs with special reference to the new imaging techniques such as cranial computed tomography and magnetic resonance imaging. These methods reveal pathognomonic patterns already very early, in association with specific clinical changes. Tuberous sclerosis must be excluded even in apparently healthy relatives by an exact diagnosis. Computed tomography is an absolutely indispensable indicator in this regard, since it is a highly sensitive method.