Motor activation in SPG4-linked hereditary spastic paraplegia.
OBJECTIVE
The aim of this study was to investigate the extent of motor cortical functional reorganisation in patients with SPG4-linked hereditary spastic paraplegia by exploring cortical motor activation related to movements of clinically affected (lower) and unaffected (upper) limbs.


METHODS
Thirteen patients and 13 normal controls matched for age, gender and handedness underwent O15-labelled water positron emission tomography during (1) right ankle flexion-extension, (2) right shoulder flexion-extension and (3) rest. Within-group comparisons of movement vs. rest (simple main effects) and between-group comparisons of movement vs. rest (group x behavioural state interaction) were performed using a random effects approach and statistical parametric mapping (SPM99).


RESULTS
Patterns of motor activation were generally comparable between groups during both tasks, although patients had a tendency towards more widespread activation in sensorimotor cortical and cerebellar regions. Statistically significant differences were restricted to the ankle movement response, however, where patients showed significantly increased regional cerebral blood flow in the right and left primary motor cortices, the supplementary motor areas and the right premotor cortex compared to controls.


CONCLUSIONS
Motor cortical reorganisation may explain this result, but as no significant differences were recognised in the motor response of the unaffected limb, differences in functional demands should also be considered.
Scheuer
Kristin H
KH
The Neurobiology Research Unit, N 9201, Copenhagen University Hospital, Denmark.
Nielsen
Jørgen E
JE
Krabbe
Katja
K
Paulson
Olaf B
OB
Law
Ian
I
D000251:Adenosine Triphosphatases / Q000235:genetics*; D000328:Adult; D000368:Aged; D001921:Brain / Q000473:pathology / Q000503:physiopathology; D001931:Brain Mapping; D002560:Cerebrovascular Circulation; D005260:Female; D007839:Functional Laterality; D006801:Humans; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000473:pathology / Q000503:physiopathology*; D009068:Movement; D009415:Nerve Net / Q000473:pathology / Q000503:physiopathology; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology*; D009473:Neuronal Plasticity / Q000502:physiology; D049268:Positron-Emission Tomography; D020127:Recovery of Function / Q000502:physiology; D015419:Spastic Paraplegia, Hereditary / Q000000981:diagnostic imaging / Q000235:genetics / Q000503:physiopathology*; D000074181:Spastin
D000251:Adenosine Triphosphatases; D000074181:Spastin; C400283:SPAST protein, human