I'm a fan of Dr. Sinan - yes, he counts as an experienced CCSVI doc!
J Neurol Sci. 1996 Dec;144(1-2):64-9.
Local cerebral blood flow in motor neuron disease: correlation with clinical findings.
Kobari M, Obara K, Watanabe S, Dembo T, Fukuuchi Y.
Local cerebral blood flow (CBF) measured by xenon-enhanced CT was correlated with the clinical findings in 21 patients with motor neuron disease (mean +/- SD age, 60 +/- 10 years). In 11 patients, CBF was also measured after intravenous injection of 2 mg thyrotropin releasing hormone (TRH). There were no significant differences in CBF of the cerebral cortex, basal ganglia, thalamus, and subcortical white matter with respect to age, duration of illness, or presence (n = 9) or absence (n = 12) of bulbar palsy. In patients with upper motor neuron disturbance (n = 10), however, local CBF in the frontal cortex was significantly lower (p < 0.05) than in those without it (n = 11). Within the frontal cortex, CBF reduction was marked in the upper posterolateral part (p < 0.05) that included the motor and premotor areas. Intravenous administration of TRH did not significantly alter the local CBF in any of the brain regions examined. An additional patient with motor neuron disease and severe dementia showed marked CBF reduction in the frontal lobe, which was in common with but much greater than the reduction in those exhibiting subcortical dementia (e.g., progressive supranuclear palsy). We conclude that in motor neuron disease patients with upper motor neuron involvement, the selective reduction of CBF in brain regions that include primary motor and premotor areas may reflect functional disturbance or neuronal degeneration in these regions. The ameliorating effect of TRH in patients with motor neuron disease, if any, appears not to be related to increases in local CBF or activation of brain function.
J Neurol Sci. 1992 Jan;107(1):19-28.
Focal reductions of cerebral blood flow in amyotrophic lateral sclerosis: a [99mTc]-d,l-HMPAO SPECT study.
Waldemar G, Vorstrup S, Jensen TS, Johnsen A, Boysen G.
We investigated regional cerebral blood flow (rCBF) using the [99mTc]-d,l-HMPAO technique with brain dedicated high resolution single photon emission computer tomography (SPECT) in 14 consecutive patients with amyotrophic lateral sclerosis (ALS), median age 62 years (45-77). Global CBF, expressed in % relative to the cerebellum, was significantly lower (P less than 0.05) in the ALS group (80.5 +/- 6.7%) than in the control group of 14 age-matched healthy volunteers (87.0 +/- 7.5%). Eight patients (57%) had abnormal rCBF distribution maps with reduced flow, primarily in the frontal lobes. Three of the 8 patients with abnormal rCBF had mild to moderate dementia and another one had mild aphasia. None of the patients with normal rCBF distribution maps had dementia. In the group of ALS patients as a whole rCBF was significantly reduced in the frontal cortex, the hippocampus, and the central white matter. We conclude that reduced rCBF, primarily in the frontal lobes, is a frequent finding in patients with ALS. The decreased rCBF may be associated with cognitive deficits and is most likely caused by neuronal degeneration and reduced metabolic needs.
Rinsho Shinkeigaku. 1992 Jan;32(1):57-61.
A case of motor neuron disease with dementia--cerebral blood flow and cerebral oxygen metabolism
Kitamura S, Taji N, Komiyama T, Sakayori O, Terashi A.
A 51-year-old man developed muscle weakness of the bilateral upper extremities, and mental changes beginning with personality change. There was no history of mental illness in his family. A neurological examination 1 year after the onset revealed muscle atrophy and fasciculation of his bilateral upper extremities Neuropsychological examination revealed concrete speech, paraphasia, and lack of judgment. Disorientation, amnesia, dyscalculia, and spatial agnosia, however, were not recognized. These neuropsychological findings were compatible with dementia of frontal lobe type. EMG and muscle biopsy revealed neurogenic muscular atrophy. There was no abnormal findings in the brain X-CT and the brain MRI. PET study using C15O2 and 15O2 revealed reduction of cerebral blood flow (CBF) and cerebral metabolic rate of oxygen (CMRO2) in the bilateral medial frontal cortex, the left temporal cortex and the bilateral thalamus. From these these findings the patient was diagnosed as having motor neuron disease with dementia. Muscle atrophy and dementia worsened gradually. A second PET study 2 years and 6 months after the onset revealed severe reduction of CBF and CMRO2 in the bilateral temporal cortex and the thalamus. These PET findings suggested that dysfunction of the temporal cortex and the thalamus related to dementia in this case.
Neurology. 2010 Mar 9;74(10):821-7. Epub 2010 Feb 10.
Gray matter perfusion correlates with disease severity in ALS.
Rule RR, Schuff N, Miller RG, Weiner MW.
OBJECTIVE: The goal of this study is to determine if regional brain perfusion, as measured by arterial spin labeling (ASL) MRI, is correlated with clinical measures of amyotrophic lateral sclerosis (ALS) disease severity. The presence of such a relationship would indicate a possible role for ASL perfusion as a marker of disease severity and upper motor neuron involvement in ALS.
METHODS: Disease severity was assessed in 16 subjects with ALS (age 54 +/- 11) using the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) and the pulmonary function measure, forced vital capacity (FVC). Upper motor neuron involvement was assessed by testing rapid tapping of the fingers and feet. Magnetic resonance perfusion images were coregistered with structural T1-weighted MRI, corrected for partial volume effects using the structural images and normalized to a study-specific atlas. Correlations between perfusion and ALS disease severity were analyzed, using statistical parametric mapping, and including age as a factor. Analyses were adjusted for multiple clusters. Result: ALS severity, as measured by the ALSFRS and FVC, was correlated with gray matter perfusion. This correlation was predominantly observed in the hemisphere contralateral to the more affected limbs. ALSFRS scores correlated with perfusion in the contralateral frontal and parietal lobe (p < 0.001) and ipsilateral frontal lobe (p < 0.02). FVC scores correlated with gray matter perfusion in contralateral frontal lobe (p < 0.001). Upper motor neuron involvement, as measured by rapid finger tapping, correlated bilaterally with perfusion in the middle cingulate gyrus (p < 0.001).
CONCLUSION: Amyotrophic lateral sclerosis (ALS) severity is correlated with brain perfusion as measured by arterial spin labeling (ASL) perfusion. This correlation appears to be independent of brain atrophy. ASL perfusion may be a useful tool for monitoring disease progression and assessing treatment effects in ALS.
Regional cerebral perfusion deficits in ALS.