Spastic Paraplegia Accompanied by Extrapyramidal Sign and Frontal Cognitive Dysfunction.

A complicated form of spastic paraplegia is a neurodegenerative disorder presenting as progressive spasticity in the bilateral lower limbs accompanied by some clinical features. The present case showed spastic paralysis and hyperreflexia in all extremities as well as lead pipe rigidity in the neck and bilateral upper extremities (R < L), decreased scores on frontal cognitive tests, a decreased accumulation of the right dorsal putamen on a DAT scan, and hypoperfusion of the bilateral frontal lobes on 99mTc-ECD single photon emission computed tomography (SPECT). The present case provides a new spectrum of spastic paraplegia based on the evidence of clinical scores and the findings of brain functional imaging.

Association of Extrapyramidal Tracts' Integrity With Performance in Fine Motor Skills After Stroke.

Background and Purpose- Tractography by diffusion tensor imaging has extended our knowledge on the contribution of damage to different pathways to residual motor function after stroke. Integrity of the corticospinal tract (CST), for example, has been identified to characterize and predict its course. Yet there is only scarce data that allow a judgment on the impact of extrapyramidal pathways between the basal ganglia on motor function poststroke. We aimed at studying their association with performance in fine motor skills after stroke. Methods- We performed probabilistic tractography and reconstructed nigro-pallidal tracts connecting substantia nigra and globus pallidus, as well as the CST in 26 healthy subjects. Resulting tracts were registered to the individual images of 20 patients 3 months after stroke, and their microstructural integrity was measured by fractional anisotropy. Clinical examination of the patients' gross (grip force) and fine (nine-hole peg test) motor skills was performed 1 year after stroke. For assessment of factors influencing nine-hole peg test, we used a multivariate model. Results- Nigro-pallidal tracts were traceable in all participants, had no overlap to the CST and passed the nucleus subthalamicus. In stroke patients, nigro-pallidal tracts ipsilateral to the stroke lesion showed a significantly reduced fractional anisotropy (ratio, 0.96±0.02; P=0.021). One year after stroke, nine-hole peg test values were significantly slower for the affected hand, while grip force was comparable between both hands. Reduced integrity of the nigro-pallidal tracts was associated with worse performance in the nine-hole peg test ( P=0.040), as was reduced integrity of the CST ( P<0.001) and younger age ( P<0.001). Conclusions- Nigro-pallidal tracts with containing connections of the nucleus subthalamicus represent a relevant part of the extrapyramidal system and specifically contribute to residual fine motor skills after stroke beyond the well-known contribution of the CST. They may deliver supportive information for prediction of motor recovery after stroke.

Recent developments in innervation imaging using iodine-123-metaiodobenzylguanidine scintigraphy in Lewy body diseases.

Radiolabeled metaiodobenzylguanidine (MIBG) is an analog of guanethidine and is taken up by the postganglionic presynaptic nerve endings. MIBG uptake in the heart correlates with adrenergic function, which can be reduced in Lewy body diseases. We described the recent developments in innervation imaging using (123)I-MIBG scintigraphy in Lewy body diseases including Parkinson's disease and dementia with Lewy bodies. Particularly, we underlined the role of MIBG scintigraphy in differential diagnosis of movement disorders. As described by recent studies, MIBG scintigraphy is a valuable diagnostic tool for differentiation between Lewy body diseases and parkinsonian syndromes or other movement disorders with parkinsonism. Furthermore, this method may provide a powerful differential diagnostic tool between dementia with Lewy bodies and Alzheimer's disease. We also reported the results of clinical investigations about the correlation between characteristics of Parkinson's disease and myocardial MIBG uptake.

D2-dopamine receptor occupancy differs between patients with and without extrapyramidal side effects.

To investigate whether the occurrence of extrapyramidal side effects was related to D2 dopamine receptor occupancy, iodobenzamide single positron emission computed tomography was carried out in 27 schizophrenic patients and 10 controls. Eighteen patients were treated with haloperidol; 9 patients were treated with clozapine. Our data suggest a relationship between D2 receptor occupancy and extrapyramidal side effects as well as the existence of a neuroleptic threshold of a striatal:frontal cortex ratio of 1.2, below which drug-induced exptrapyramidal side effects can be expected.