-
U Rüb,
K Gierga,
E R Brunt,
R A I de Vos, M Bauer,
L Schöls,
K Bürk,
G Auburger,
J Bohl,
C Schultz,
M Vuksic,
G J Burbach,
H Braak,
T Deller
[show abstract]
[hide abstract]
ABSTRACT: The pre-cerebellar nuclei act as a gate for the entire neocortical, brainstem and spinal cord afferent input destined for the cerebellum. Since no pathoanatomical studies of these nuclei had yet been performed in spinocerebellar ataxia type 2 (SCA2) or type 3 (SCA3), we carried out a detailed postmortem study of the pre-cerebellar nuclei in six SCA2 and seven SCA3 patients in order to further characterize the extent of brainstem degeneration in these ataxic disorders. By means of unconventionally thick serial sections through the brainstem stained for lipofuscin pigment and Nissl material, we could show that all of the pre-cerebellar nuclei (red, pontine, arcuate, prepositus hypoglossal, superior vestibular, lateral vestibular, medial vestibular, interstitial vestibular, spinal vestibular, vermiform, lateral reticular, external cuneate, subventricular, paramedian reticular, intercalate, interfascicular hypoglossal, and conterminal nuclei, pontobulbar body, reticulotegmental nucleus of the pons, inferior olive, and nucleus of Roller) are among the targets of both of the degenerative processes underlying SCA2 and SCA3. These novel findings are in contrast to the current neuropathological literature, which assumes that only a subset of pre-cerebellar nuclei in SCA2 and SCA3 may undergo neurodegeneration. Widespread damage to the pre-cerebellar nuclei separates all three phylogenetically and functionally defined regions of the cerebellum, impairs their physiological functions and thus explains the occurrence of gait, stance, limb and truncal ataxia, dysarthria, truncal and postural instability with disequilibrium, impairments of the vestibulo-ocular reaction and optokinetic nystagmus, slowed and saccadic smooth pursuits, dysmetrical horizontal saccades, and gaze-evoked nystagmus during SCA2 and SCA3.
Acta Neurovegetativa 12/2005; 112(11):1523-45. · 2.73 Impact Factor
-
U. Rüb,
K. Gierga,
E. R. Brunt,
R. A. I. de Vos, M. Bauer,
L. Schöls,
K. Bürk,
G. Auburger,
J. Bohl,
C. Schultz,
M. Vuksic,
G. J. Burbach,
H. Braak,
T. Deller
[show abstract]
[hide abstract]
ABSTRACT: The precerebellar nuclei act as a gate for the entire neocortical, brainstem and spinal cord afferent input destined for the
cerebellum. Since no pathoanatomical studies of these nuclei had yet been performed in spinocerebellar ataxia type 2 (SCA2)
or type 3 (SCA3), we carried out a detailed postmortem study of the precerebellar nuclei in six SCA2 and seven SCA3 patients
in order to further characterize the extent of brainstem degeneration in these ataxic disorders. By means of unconventionally
thick serial sections through the brainstem stained for lipofuscin pigment and Nissl material, we could show that all of the
precerebellar nuclei (red, pontine, arcuate, prepositus hypoglossal, superior vestibular, lateral vestibular, medial vestibular,
interstitial vestibular, spinal vestibular, vermiform, lateral reticular, external cuneate, subventricular, paramedian reticular,
intercalate, interfascicular hypoglossal, and conterminal nuclei, pontobulbar body, reticulotegmental nucleus of the pons,
inferior olive, and nucleus of Roller) are among the targets of both of the degenerative processes underlying SCA2 and SCA3.
These novel findings are in contrast to the current neuropathological literature, which assumes that only a subset of precerebellar
nuclei in SCA2 and SCA3 may undergo neurodegeneration. Widespread damage to the precerebellar nuclei separates all three phylogenetically
and functionally defined regions of the cerebellum, impairs their physiological functions and thus explains the occurrence
of gait, stance, limb and truncal ataxia, dysarthria, truncal and postural instability with disequilibrium, impairments of
the vestibulo-ocular reaction and optokinetic nystagmus, slowed and saccadic smooth pursuits, dysmetrical horizontal saccades,
and gaze-evoked nystagmus during SCA2 and SCA3.
Acta Neurovegetativa 10/2005; 112(11):1523-1545. · 2.73 Impact Factor
-
K Gierga,
K Bürk, M Bauer,
G Orozco Diaz,
G Auburger,
C Schultz,
M Vuksic,
L Schöls,
R A I de Vos,
H Braak,
T Deller,
U Rüb
[show abstract]
[hide abstract]
ABSTRACT: Although the cranial nerves, their nuclei and related fiber tracts are crucial for a variety of oculomotor, somatomotor, somatosensory, auditory, vestibular-related, autonomic and ingestion-related functions, knowledge regarding the extent of their involvement in spinocerebellar ataxia type 2 (SCA2) patients is incomplete. Accordingly, we performed a pathoanatomical analysis of these structures in six clinically diagnosed SCA2 patients. Unconventionally thick serial sections through the brainstem stained for lipofuscin pigment (aldehyde-fuchsin) and Nissl material (Darrow red) showed that all oculomotor, somatomotor, somatosensory, auditory, vestibular and autonomic cranial nerve nuclei may undergo neurodegeneration during SCA2. Similarly, examination of myelin-stained thick serial sections revealed that nearly all cranial nerves and associated fiber tracts may sustain atrophy and myelin loss in SCA2 patients. In view of the known functional role of the affected cranial nerves, their nuclei and associated fiber tracts, the present findings provide appropriate pathoanatomical explanations for some of the disease-related and unexplained symptoms seen in SCA2 patients: double vision, gaze palsy, slowing of saccades, ptosis, ingestion-related malfunctions, impairments of the optokinetic nystagmus and the vestibulo-ocular reaction, facial and tongue fasciculation-like movements, impaired centripetal transmission of temperature-related information from the face, dystonic posture of the neck, as well as abnormalities of the brainstem auditory evoked potentials.
Acta Neuropathologica 07/2005; 109(6):617-31. · 9.32 Impact Factor
