We are analyzing https://link.springer.com/article/10.1007/s12311-010-0235-0.

Title:
The Neuropathology of Late-Onset Friedreich’s Ataxia | The Cerebellum
Description:
Friedreich’s ataxia (FRDA) affects very young persons. In a large series, the mean ages of onset and death were 11 and 38 years, respectively. The clinical spectrum of FRDA has expanded after genetic confirmation of the mutation became a routine laboratory test. The main cause of death in juvenile-onset FRDA is cardiomyopathy whereas patients with late-onset are more likely to succumb to neurological disability or an intercurrent illness. Many patients with early onset now survive for 20 years or longer. This study made a systematic comparison of the neuropathology in 14 patients with juvenile onset and long survival, and five patients with late onset and long survival. Mean ages of onset (± standard deviation) were 10 ± 5 and 28 ± 13 years, respectively. Disease durations were 33 ± 11 and 47 ± 11 years, respectively. Cross-sectional areas of the thoracic spinal cord were greatly reduced from the normal state but did not differ between the two groups. Similarly, the neurons of dorsal root ganglia were significantly reduced in size in both juvenile- and late-onset cases of FRDA. The dentate nucleus showed severe loss of neurons as well as modification and destruction of corticonuclear terminals in all FRDA patients. Delayed atrophy of the dentate nucleus is the likely cause of the ataxic phenotype of FRDA in late-onset cases, but the reason for the delay is unknown. Frataxin levels in the dentate nucleus of two patients with late onset were similar to those of seven patients with juvenile onset.
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Keywords {🔍}

article, ataxia, google, scholar, friedreichs, pubmed, cas, onset, koeppen, patients, research, albany, usa, frda, clinical, disease, dentate, nucleus, medical, privacy, cookies, content, cerebellum, lateonset, arnulf, access, neurol, publish, search, neuropathology, morral, years, spinal, cord, neurons, dorsal, root, friedreich, center, data, information, log, journal, mccomb, genetic, early, juvenile, survival, late, ganglia,

Topics {✒️}

month download article/chapter dorsal root ganglion privacy choices/manage cookies full article pdf dorsal root ganglia related subjects va medical center nebraska medical center late-onset cases european economic area routine laboratory test cross-sectional areas thoracic spinal cord left ventricular hypertrophy cranial spinal cord mr findings involving le quan kh neurol neurosurg psychiatry ataxia research alliance conditions privacy policy mri-confirmed atrophy iron-responsive proteins selective iron chelation promotes cell survival accepting optional cookies ataxia selectively involves national ataxia foundation early diagnostic criteria article koeppen albany medical college late-onset friedreich journal finder publish disease durations disease phenotype dentate nucleus cambridge university press article log author information authors main content log anat rec juvenile-onset frda neurol sci check access instant access article cite cerebellum article normal state frataxin levels extramitochondrial frataxin spinal cord

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         description:Friedreich’s ataxia (FRDA) affects very young persons. In a large series, the mean ages of onset and death were 11 and 38 years, respectively. The clinical spectrum of FRDA has expanded after genetic confirmation of the mutation became a routine laboratory test. The main cause of death in juvenile-onset FRDA is cardiomyopathy whereas patients with late-onset are more likely to succumb to neurological disability or an intercurrent illness. Many patients with early onset now survive for 20 years or longer. This study made a systematic comparison of the neuropathology in 14 patients with juvenile onset and long survival, and five patients with late onset and long survival. Mean ages of onset (± standard deviation) were 10 ± 5 and 28 ± 13 years, respectively. Disease durations were 33 ± 11 and 47 ± 11 years, respectively. Cross-sectional areas of the thoracic spinal cord were greatly reduced from the normal state but did not differ between the two groups. Similarly, the neurons of dorsal root ganglia were significantly reduced in size in both juvenile- and late-onset cases of FRDA. The dentate nucleus showed severe loss of neurons as well as modification and destruction of corticonuclear terminals in all FRDA patients. Delayed atrophy of the dentate nucleus is the likely cause of the ataxic phenotype of FRDA in late-onset cases, but the reason for the delay is unknown. Frataxin levels in the dentate nucleus of two patients with late onset were similar to those of seven patients with juvenile onset.
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