Health Programs paints a different picture. In a retrospective case control study, Paris and Canavan compared 32 anesthesiologists with 36 physician controls, and after an average follow-up of 7.5 yr, there was no difference in the relapse rates between these 2 groups. Likewise, the outcomes of residents did not differ from attending physicians. A similar report from Pelton involving 255 physic...

The leucodystrophies are a heterogeneous group of inherited neurological disorders all characterized by progressive symmetrical demyelination without any marked inflam-matory response, causing loss of motor, sensory, and intellectual functions with an eventual fatal outcome. Included in the group are metachromatic leucodystrophy (sulphatide lipidosis), Krabbe's globoid cell leucodystrophy, Cana...

BACKGROUND There is a high incidence of hereditary degenerative diseases of the central nervous system in purebred dogs. HYPOTHESIS Cerebellar ataxia in Malinois puppies, caused by degenerative changes that predominate in cerebellar nuclei and the granular cell layer, is a hereditary disorder that is distinct from cerebellar cortical abiotrophies. ANIMALS Thirteen Malinois puppies with cere...

The Editorial Board advises the editorial team on all important issues and are selected by the Board of the Gesellschaft Deutscher Chemiker (GDCh; German Chemical Society) upon recommendation by the Editorial Board and the editorial team. After 19 years on the Editorial Board (and 10 years as its Chairman) FranÅois Diederich will now join the International Advisory Board (see below). Hartmut Mi...

Spongy degeneration with cerebellar ataxia (SDCA) is a severe neurodegenerative disease with monogenic autosomal recessive inheritance in Malinois dogs, one of the four varieties of the Belgian Shepherd breed. We performed a genetic investigation in six families and seven isolated cases of Malinois dogs with signs of cerebellar dysfunction. Linkage analysis revealed an unexpected genetic hetero...

A retrospective study was carried out to define the spectrum of spontaneous diseases in ostriches and few other captive ratites, order Struthioniformes, in northwestern Germany. The investigation included 71 ratites necropsied between 1968 and 2014. They consisted of 54 ostriches, 5 emus, and 12 rheas with 37 adults, 23 juveniles and 11 neonates and embryonated eggs. Necropsy reports were revie...

A model animal showing spontaneous onset is a useful tool for investigating the mechanism of disease. Here, I would like to introduce two aging model animals expected to be useful for neuroscience research: the senescence-accelerated mouse (SAM) and the klotho mouse. The SAM was developed as a mouse showing a senescence-related phenotype such as a short lifespan or rapid advancement of senescen...

Vuia1 reported two twin brothers diagnosed with congenital spongy degeneration of the brain featured by accumulation of lamellar bodies within the mitochondria, and free in the cellular cytoplasm with tendency to form inclusions of the multilamellar or finger-print type. Watanabe2 described in a 51/2-year-old boy with congenital myopathy associated with communicating hydrocephalus myofibrillar ...

MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke) syndrome was first described in 1984 by Pavlakis et al. [1] after reporting three cases of their own and identifying eight others in the literature. Although MELAS syndrome shares some similarities with Kearns-Sayre syndrome and MERRF (myoclonus epilepsy and ragged-red fibers) syndrome, they believe it to be a distinct ...

Creutzfeldt-Jacob disease (CJD) is a rare and transmissible neurological disorder, which has been increasing in frequency in the United States over the past two decades [1]. CJD is a spongiform encephalopathy characterized by a conformational change of prion protein [2]. The death rate for CJD in the USA is 0.9 per million [1]. We report 7 cases of CJD from North Shore University Hospital, a co...