- Email: [email protected]
15. PET and multiple lacunae: relation between localization, regional cerebral blood flow and vasocapacitance
15. PET and multiple lacunae: relation between localization, regional cerebral blood flow and vasocapacitance - D. Decoo, J. De Reuck, P. Goethals and I. Lemahieu, PET Pm,jrct. Stutc lJuiwr:rity.
Upadhyaya,
Ghcwt. Belgiwn
The relation between multiple lacunae and cognitive deterioration is not clear. In theory there are 2 possible connections. The dementia can be due to a disconnection between cortical and subcortical structures, without vascular problems in the disconnected region. Otherwise the dementia can be a manifestation of extensive vascular insufficiency in the cortical structures. In both situations there is a hypometabolism over the cortical regions, with an associated lower cerebral blood flow (rCBF). If the local vascular system is affected, a decrease of the vasocapacitance is also expected. To answer this problem a PET investigation with ‘jNH1 and acetazolamide (Diamox@) was performed in 61 patients. This technique evaluates the regional CBF and vasocapacitance. There were 2 patient groups with multiple infarcts with and without dementia and a control group. Our study showed a decreased ICBF over the cortical regions with a normal vasocapacitance, while rCBF and vasocapacitance were both significantly decreased over the thalamus. Multiple-infarct dementia is the consequence of cortical disconnection caused by lacunar lesions in the thalamus.
16. Quality of life scales and neurological dysfunction in brain tumor patients - H.B.C. Verbiest. A. Hovestadt, W.L.J. van Putten and C.J. Vecht, L)r: Darlic~l detl Hoed Kliniek.
17. Regional mapping of the facioscapulohumeral muscular dystrophy gene on 4q35: linkage analysis of the International Consortium - G.W. Padberg. C. Wi.jmenga, M. B. Weiffenbach,
O.F. Brouwer, J. Murray, M.
PericaJ-Vance, P. Lunt, R.R. Frants, P.S. Harper and M. Sarfarazi, Depur-tmcnt ofNrur_olo,qg. Stutr Uniwrsit~. Lri&n. T/w Nethwland.s
The International Consortium for linkage studies in facie+ capulohumeral muscular dystrophy (FMD) combined the data from 6 centers to determine the order of 4 marker loci relative IO the FMD locus in the chromosome region 4q35. In 65 families with SO4 affected and 559 non-affected
individuals
307X gent)-
types were studied. The mean number of informative meioses for each of the markers in the combined data was 64X. By use of the CRIMAP program the most likely order of the loci is: CenD4S I7 I -FI I -D4S 163.D4S 139.FMD-Tel. The area covered by the 5 loci is estimated to be 2.53 CM in males and 13.X CM in females with a sex average of 19.5 CM. The excess in the male recombination fraction was statistically significant (P = 0.0013). Comparison of the two-point and multipoint lod scores in the FMD familics showed no evidence for heterogeneity of this disorder. The loci D4Sl3Y and D4S I63 were identified as the ones closest linked. with 99%) upper confidence intervals of X and 10% recombinanls, respectively. Being on the same side of the FMD locus. thc\e markers are as yet not suitable for presymptomatic
diagnosis.
Rotter-
tkm~. 771~ Ncthcrlatd\
1X. Polymyositis
and dermatomyositis:
concept for a diagnos-
tic protocol - P.J.H. Jongen, Nrlrr~~Io,~ic~u/I~rstitrctc.St. RodQuality of life scales are important for monitoring therapy in oncology, but their use has not been validated in brain tumor patients. We compared 3 easily operable activities of daily life (ADL) scales (Karnofsky); Rankin (St,nk, I9 (1988) 604-607): Ebrahim (./. E/~iclcn~iol. C‘ommun. Hdth. 39 (I 985) 86-89): and a modified Barer scale (Neur.o~~idcmio/o,~?, 7 (I 988) l-l 2) for rating neurological dysfunction. The Barer scale is divided into a functional scale and a neurological scale with I2 items with subscores for cognition, hemisphere. and posterior fossa lesions. We prospectively examined 57 patient5 with CT documented brain metastases. on days 0. 7 and 28 following start of treatment. The 3 ADL scales were strongly correlated, showing correlation coefficients I. %.X0, and factor analysis revealed I underlying common factor explaining 89% of the variance. The functional Barer sum-score and its itema for ambulation and dressing correlated strongly with the other ADL scales (I’ 10.70). The sum-score of the neurological Barer and sub-scores for cognition, hemisphere and posterior fossa function correlated weakly with ADL scales and functional Barer (I. 0.2(-O.S()). There was a good correlation between cognition and hemisphere function (0.64), but no significant correlations
between these and posterior fossa score.
We conclude that Rankin. Ebrahim and functional Barer scales do not add extra information compared to Kamofsky. The neurological Barer scale measures other dimensions than ADL function and seems a valuable addition to the Kamofsky scale for assessment of severity of specific neurological dysfunction in brain tumor patients.
horrd Hnspl’itd.
N~jnwgcrr. The NC~~CI~INII~Y
Diagnosis of polymyositis
(PM) and dermatomyositis
(DM I is
based on clinical symptoms and signs. laboratory tests, elcctroneuromyography (EMG), and results from muscle biopsy. In 197.5 Bohan and Peter established diagnostic criteria for definitive. probable, and possible PM and DM. Recently. Dalakas formulated revised criteria. We propose a concept for the diagnostic work-up of patients with PM or DM. Three phases of examination are delineated and criteria are proposed for each phase. First. history and neurological, internal. rheumatological, and dermatologlcal examinations
(progressive
proximal
muscle
weakness, dennal
changes). Second. laboratory tests (increase of serum CK, cxclumyopathies). and EMG (myopathic Third. muscle biopsy for histological examination (inflammation. necrosis; exclusion of non-inllammatory myopathies). The protocol also provides guidelines for additional sion of non-inflammatory
changes).
evaluation
of patients with definitive
or probable PM or CIM,
including dynamometry (quantification of muscle weakness). ECG and lung function test, screening for an associated autoinmune disorder and for an underlying neoplasm.
19. Spastic paraplegia due to a spinal vascular malformation in Rendu Osler Weber disease - J. Leendcrs. M.K. Viaene and H. Carton. &pur-tmwt of‘Ncr4rcdo,y~. Uiwmity Hospittrl
Upadhyaya,
Ghcwt. Belgiwn
The relation between multiple lacunae and cognitive deterioration is not clear. In theory there are 2 possible connections. The dementia can be due to a disconnection between cortical and subcortical structures, without vascular problems in the disconnected region. Otherwise the dementia can be a manifestation of extensive vascular insufficiency in the cortical structures. In both situations there is a hypometabolism over the cortical regions, with an associated lower cerebral blood flow (rCBF). If the local vascular system is affected, a decrease of the vasocapacitance is also expected. To answer this problem a PET investigation with ‘jNH1 and acetazolamide (Diamox@) was performed in 61 patients. This technique evaluates the regional CBF and vasocapacitance. There were 2 patient groups with multiple infarcts with and without dementia and a control group. Our study showed a decreased ICBF over the cortical regions with a normal vasocapacitance, while rCBF and vasocapacitance were both significantly decreased over the thalamus. Multiple-infarct dementia is the consequence of cortical disconnection caused by lacunar lesions in the thalamus.
16. Quality of life scales and neurological dysfunction in brain tumor patients - H.B.C. Verbiest. A. Hovestadt, W.L.J. van Putten and C.J. Vecht, L)r: Darlic~l detl Hoed Kliniek.
17. Regional mapping of the facioscapulohumeral muscular dystrophy gene on 4q35: linkage analysis of the International Consortium - G.W. Padberg. C. Wi.jmenga, M. B. Weiffenbach,
O.F. Brouwer, J. Murray, M.
PericaJ-Vance, P. Lunt, R.R. Frants, P.S. Harper and M. Sarfarazi, Depur-tmcnt ofNrur_olo,qg. Stutr Uniwrsit~. Lri&n. T/w Nethwland.s
The International Consortium for linkage studies in facie+ capulohumeral muscular dystrophy (FMD) combined the data from 6 centers to determine the order of 4 marker loci relative IO the FMD locus in the chromosome region 4q35. In 65 families with SO4 affected and 559 non-affected
individuals
307X gent)-
types were studied. The mean number of informative meioses for each of the markers in the combined data was 64X. By use of the CRIMAP program the most likely order of the loci is: CenD4S I7 I -FI I -D4S 163.D4S 139.FMD-Tel. The area covered by the 5 loci is estimated to be 2.53 CM in males and 13.X CM in females with a sex average of 19.5 CM. The excess in the male recombination fraction was statistically significant (P = 0.0013). Comparison of the two-point and multipoint lod scores in the FMD familics showed no evidence for heterogeneity of this disorder. The loci D4Sl3Y and D4S I63 were identified as the ones closest linked. with 99%) upper confidence intervals of X and 10% recombinanls, respectively. Being on the same side of the FMD locus. thc\e markers are as yet not suitable for presymptomatic
diagnosis.
Rotter-
tkm~. 771~ Ncthcrlatd\
1X. Polymyositis
and dermatomyositis:
concept for a diagnos-
tic protocol - P.J.H. Jongen, Nrlrr~~Io,~ic~u/I~rstitrctc.St. RodQuality of life scales are important for monitoring therapy in oncology, but their use has not been validated in brain tumor patients. We compared 3 easily operable activities of daily life (ADL) scales (Karnofsky); Rankin (St,nk, I9 (1988) 604-607): Ebrahim (./. E/~iclcn~iol. C‘ommun. Hdth. 39 (I 985) 86-89): and a modified Barer scale (Neur.o~~idcmio/o,~?, 7 (I 988) l-l 2) for rating neurological dysfunction. The Barer scale is divided into a functional scale and a neurological scale with I2 items with subscores for cognition, hemisphere. and posterior fossa lesions. We prospectively examined 57 patient5 with CT documented brain metastases. on days 0. 7 and 28 following start of treatment. The 3 ADL scales were strongly correlated, showing correlation coefficients I. %.X0, and factor analysis revealed I underlying common factor explaining 89% of the variance. The functional Barer sum-score and its itema for ambulation and dressing correlated strongly with the other ADL scales (I’ 10.70). The sum-score of the neurological Barer and sub-scores for cognition, hemisphere and posterior fossa function correlated weakly with ADL scales and functional Barer (I. 0.2(-O.S()). There was a good correlation between cognition and hemisphere function (0.64), but no significant correlations
between these and posterior fossa score.
We conclude that Rankin. Ebrahim and functional Barer scales do not add extra information compared to Kamofsky. The neurological Barer scale measures other dimensions than ADL function and seems a valuable addition to the Kamofsky scale for assessment of severity of specific neurological dysfunction in brain tumor patients.
horrd Hnspl’itd.
N~jnwgcrr. The NC~~CI~INII~Y
Diagnosis of polymyositis
(PM) and dermatomyositis
(DM I is
based on clinical symptoms and signs. laboratory tests, elcctroneuromyography (EMG), and results from muscle biopsy. In 197.5 Bohan and Peter established diagnostic criteria for definitive. probable, and possible PM and DM. Recently. Dalakas formulated revised criteria. We propose a concept for the diagnostic work-up of patients with PM or DM. Three phases of examination are delineated and criteria are proposed for each phase. First. history and neurological, internal. rheumatological, and dermatologlcal examinations
(progressive
proximal
muscle
weakness, dennal
changes). Second. laboratory tests (increase of serum CK, cxclumyopathies). and EMG (myopathic Third. muscle biopsy for histological examination (inflammation. necrosis; exclusion of non-inllammatory myopathies). The protocol also provides guidelines for additional sion of non-inflammatory
changes).
evaluation
of patients with definitive
or probable PM or CIM,
including dynamometry (quantification of muscle weakness). ECG and lung function test, screening for an associated autoinmune disorder and for an underlying neoplasm.
19. Spastic paraplegia due to a spinal vascular malformation in Rendu Osler Weber disease - J. Leendcrs. M.K. Viaene and H. Carton. &pur-tmwt of‘Ncr4rcdo,y~. Uiwmity Hospittrl