Involvement of the cranial nerves and their nuclei in spinocerebellar ataxia type 2 (SCA2) View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2005-05-19

AUTHORS

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

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. More... »

PAGES

617-631

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00401-005-1014-8

DOI

http://dx.doi.org/10.1007/s00401-005-1014-8

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1026525315

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/15906049


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28 schema:description 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.
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35 schema:keywords Nissl material
36 SCA2 patients
37 abnormalities
38 affected cranial nerve
39 analysis
40 appropriate pathoanatomical explanations
41 ataxia type 2
42 ataxia type 2 (SCA2) patients
43 atrophy
44 auditory
45 autonomic cranial nerve nuclei
46 brainstem
47 brainstem auditory
48 centripetal transmission
49 cranial nerve nuclei
50 cranial nerves
51 double vision
52 dystonic posture
53 examination
54 examination of myelin
55 explanation
56 extent
57 face
58 fasciculation-like movements
59 fiber tracts
60 findings
61 function
62 functional role
63 impairment
64 information
65 ingestion-related functions
66 ingestion-related malfunctions
67 involvement
68 knowledge
69 lipofuscin pigment
70 loss
71 malfunction
72 materials
73 movement
74 myelin
75 myelin loss
76 neck
77 nerve
78 nerve nuclei
79 neurodegeneration
80 nucleus
81 nystagmus
82 oculomotor
83 optokinetic nystagmus
84 palsy
85 pathoanatomical analysis
86 pathoanatomical explanation
87 patients
88 pigments
89 posture
90 potential
91 present findings
92 ptosis
93 reaction
94 related fiber tracts
95 role
96 saccades
97 sections
98 serial sections
99 slowing
100 slowing of saccades
101 somatomotor
102 somatosensory
103 spinocerebellar ataxia type 2
104 spinocerebellar ataxia type 2 patients
105 structure
106 symptoms
107 temperature-related information
108 thick serial sections
109 tongue fasciculation-like movements
110 tract
111 transmission
112 type 2
113 type 2 patients
114 unexplained symptoms
115 variety
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118 view
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