Prolonged cortical relay time of long latency reflex and central motor conduction in patients with spinocerebellar ataxia type 6

Spinocerebellar ataxia type 6 (SCA6): neurodegeneration goes beyond the known brain predilection sites

“…Along with the well‐known predilection sites (that is, cerebellar cortex, dentate nucleus and inferior olive) [7,8,10–15], the neuropathological process involved the cerebellar fastigial, globose and emboliform nuclei, the allocortex and neocortex, the thalamus, the midbrain dopaminergic system, as well as a variety of oculomotor, auditory, vestibular, ingestion‐related, somatosensory and precerebellar brainstem nuclei. These novel findings confirm recent clinical symptom‐based assumptions that brain damage in SCA6 may go beyond the well‐known cerebellar and brainstem predilection sites [3,5,7,9,19] and that the substantia nigra and the pyramidal system may be affected in SCA6 [16,18,20]. Although in the SCA6 patients studied here, the extent of brain damage displayed some overlap with those commonly seen in terminal SCA2, SCA3 and SCA7 patients, it was less pronounced and, in contrast to terminal SCA2, SCA3 and SCA7 patients, did not include atrophy of central nervous fibre tracts or extracerebellar myelin loss [21,25,42–49].…”

Regional patterns of cerebral glucose metabolism in spinocerebellar ataxia type 2, 3 and 6

“…By transcranial magnetic stimulation, abnormalities in motor cortex activation have also been observed in patients with SCA1, SCA2, SCA3, Friedreich ataxia [26], and SCA6 [6,18,26].…”

“…Sagittal, coronal, and transverse glass brain images and SPM projection images on normalized MRI slices illustrating regions of decreased18 F-FDG metabolism in SCA subtypes. (P < 0.001, uncorrected) (A) SCA2 (P < 0.001, uncorrected): the cerebellum, pons, parahippocampal gyrus and frontal cortex are affected.…”

Long-latency reflexes in patients with Behçet's disease

“…Only a mild involvement of this pathway might not be sufficient to cause abnormalities in amplitude ratios of these potentials. The amplitude of LLR2 was also not changed in spite of prolonged latency of this component in some degenerative conditions of CNS [18]. Therefore, it seems that the reduction in the number of corticospinal fibers may cause a mild increase in LLR2 latency.…”