HTLV-I-associated myelopathy (HAM/TSP) is the most common neurological manifestation of HTLV-I, causing progressive weakness, sensory disturbance, and sphincter dysfunction. Although motor disorders have been well described, few studies have associated cognitive disorders and HTLV-I infection. In areas endemic for HTLV-I infection, the differential diagnosis between HAM/TSP and other myelopathy...

The radiologic evolution of Leigh disease is documented with sequential brain MR in the acute phase of the illness, at 3 weeks, and at 3 months. High-signal-intensity lesions seen on T2-weighted images in the first week resolved by 3 months, whereas new lesions appear during the chronic stage. Putamenal involvement is not a pathognomonic radiologic finding. Brain stem tegmentum, particularly th...

Neuronal vacuolation and spinocerebellar degeneration is a rare, presumably inherited condition that is reported only in Rottweilers and in crossbred dogs with known or potential Rottweiler heritage. Gross and histopathologic findings include laryngeal muscle atrophy, neuronal vacuolation, and a combined central and peripheral axonopathy. Two 6-month-old Boxer puppies from the same litter were ...

Chronic traumatic encephalopathy (CTE) has been linked to participation in contact sports such as boxing and American football. CTE results in a progressive decline of memory and cognition, as well as depression, suicidal behavior, poor impulse control, aggressiveness, parkinsonism, and, eventually, dementia. In some individuals, it is associated with motor neuron disease, referred to as chroni...

Cranial CT changes associated with metabolic and storage disorders have been well documented. However, until recently [1], CT findings in galactosemia had not been shown. Previous neuropathologic autopsy findings in galactosemia have been described by Crome [2] as a "microencephaly caused by a mainly burnt out gliotic encephalomyelopathy ." Hypothesized causes for this encephalopathy include hy...

UNLABELLED Leigh syndrome (LS), or subacute necrotizing encephalomyelopathy, having relatively homogeneous clinical symptomatology and pattern of neuropathological changes, shows remarkable heterogeneity in biochemical and molecular background. G8363A mitochondrial DNA mutation typical for MERRF syndrome and progressive cardiomyopathy may also be associated with LS. Clinical, biochemical and pa...

Leigh syndrome (LS) is a subacute necrotizing encephalomyelopathy with gliosis in several brain regions that usually results in infantile death. Loss of murine Ndufs4, which encodes NADH dehydrogenase (ubiquinone) iron-sulfur protein 4, results in compromised activity of mitochondrial complex I as well as progressive neurodegenerative and behavioral changes that resemble LS. Here, we report the...