Clinical features and SPECT scanning in presumed cortico-basal ganglionic degeneration

doi:10.1016/0967-5868(95)90053-5

Journal of Clinical Neuroscience

Volume 2, Issue 4, October 1995, Pages 321-328

https://doi.org/10.1016/0967-5868(95)90053-5 Get rights and content

Abstract

Eight patients presented with a strikingly similar core syndrome of asymmetric upper limb rigidity and dystonia (which was maximal in the non-dominant limb in seven), accompanied by ipsilateral ideomotor apraxia, cortical sensory disturbane, and sensory reflex myoclonus. The clinical diagnosis in each case was of presumed cortico-basal ganglionic degeneratioan. The clinical features in our cases matched those of other series closely, except for a higher prevalence of dementia (presumably explained by referral bias), and a lower incidence of oculomotor abnormalities. Seven of the eight patients underwent ⁵⁹Tc-HMPAO SPECT scanning, which in each case revealed contralateral fronto-parietal perfusion defects out of proportion to the focal or generalised atrophy demonstrated on MRI scans. SPECT scanning appears to be a useful confirmatory investigation in patients presenting with this uncommon but distinctive clinical syndrome, and in separating them from patients with other causes of asymmetric rigid syndromes, such as Parkinsons's disease.

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Cited by (7)

Contribution of cerebral perfusion scintigraphy in the exploration of cortico-basal syndrome
2015, Medecine Nucleaire
Le syndrome cortico-basal (SCB), syndrome neurologique complexe d’origine dégénérative, peut avoir plusieurs étiologies dont la dégénérescence cortico-basale (DCB) et la maladie d’Alzheimer (MA). L’objectif de cette étude était d’étudier les anomalies de perfusion cérébrale en fonction du profil des biomarqueurs (BM) du LCR dans le SCB.
Quinze patients répondant aux critères cliniques de SCB ont eu une scintigraphie cérébrale de perfusion au ^99mTc-ethyl cysteinate dimer (^99mTc-ECD) (740 à 925 MBq) et un dosage des biomarqueurs (Aβ42, tau et p-tau) dans le LCR. Les scintigraphies cérébrales de plusieurs sous-groupes de sujets ont été comparées avec le logiciel SPM8 : 1) 5 patients présentant un SCB avec un profil des biomarqueurs en faveur de MA (SCB-MA) ; 2) 10 patients présentant un SCB avec un profil des biomarqueurs écartant la MA (SCB-nonMA) ; 3) 24 témoins sains (Tem-N) ; 4) 24 patients présentant une forme typique amnésique de MA (Tem-MA).
Comparé au groupe Tem-N, le groupe SCB entier (non MA et MA) présentait une hypoperfusion corticale asymétrique prédominant au niveau du cortex pariéto-frontal gauche, le groupe SCB-MA une hypoperfusion relativement symétrique prédominant dans le cortex associatif postérieur et le groupe SCB-nonMA une hypoperfusion plus asymétrique avec une atteinte périrolandique, préfrontale et périsylvienne gauche. La comparaison avec les Tem-MA ne retrouvait, pour le groupe SCB-MA, pas de différence significative et, pour le groupe SCB-nonMA, une hypoperfusion périrolandique bilatérale asymétrique.
Dans les SCB liés à la MA, l’hypoperfusion est symétrique et prédomine dans le cortex associatif postérieur, alors que dans les SCB non MA, l’hypoperfusion est plus asymétrique et antérieure avec une atteinte périrolandique.
The cortico-basal syndrome (CBS), a complex clinical neurodegenerative disorder, may have multiple aetiologies including corticobasal degeneration (CBD) and Alzheimer's disease (AD). The aim of this study was to explore the brain perfusion profile in CBS patients according to the biological profile of CFS biomarkers (BM).
Fifteen patients fulfilling clinical criteria of CBS underwent a brain perfusion scintigraphy with ^99mTc-ECD and a lumbar punction to determine the biochemical profile of CSF biomarkers (BM). Several groups were compared with SPM8 software: 1) 5 patients with SCB and with an AD CSF profile (CBS-AD); 2) 10 patients with CBS and not having an AD CSF profile (SCB-nonAD); 3) 24 healthy controls (Wit-N); 4) 24 typical amnestic (AD).
Compared to the group Wit-N, the entire SCB group (non-AD and AD) showed an asymmetric cortical hypoperfusion with the left parietal-frontal cortex predominance, the SCB-AD group a symmetrical hypoperfusion in the association posterior cortex and the SCB-nonAD group a more asymmetrical hypoperfusion with a peri-rolandic, prefrontal and left perisylvian involvement. The comparisons with the typical amnestic AD found no significant difference for the SCB-AD group and, for the SCB-nonAD group, an asymmetric bilateral peri-rolandic hypoperfusion.
For the SCB related to AD, the hypoperfusion is symmetrical and mainly in the association posterior cortex, whereas for the SCB non-related to AD pathology, the hypoperfusion is more asymmetrical and anterior with a peri-rolandic involvement.
Classification of parkinsonian syndromes via factorial discriminant analysis of brain SPECT data
2009, Revue Neurologique
Malgré l’existence de critères cliniques bien définis, le diagnostic des maladies dégénératives de l’adulte marquées par un syndrome parkinsonien est parfois mis en échec. La distinction entre paralysie supranucléaire progressive (PSP) et dégénérescence corticobasale (DCB) notamment peut-être difficile. L’objectif de cette étude était d’évaluer l’apport du traitement par analyse factorielle discriminante (AFD) de données issues de l’imagerie fonctionnelle cérébrale.
Soixante-deux patients furent répartis en trois groupes selon des critères cliniques : maladie de Parkinson (MP), PSP et DCB. Des index de fixation du traceur de perfusion (le ^99mTc-HmPaO) et des index d’asymétrie furent déterminés à partir de mesures effectuées dans 13 paires de régions d’intérêt par tomoscintigraphie d’émission monophotonique (TEMP). Ces données firent l’objet d’une AFD afin de déterminer si elles permettaient de répartir les patients dans les mêmes groupes que ceux établis selon les données cliniques.
Cette approche statistique permettait de distinguer clairement MP, PSP et DCB puisque l’AFD classait correctement tous les patients. Les régions frontale médiane, temporopariétale et pariétale étaient les plus discriminantes.
L’utilisation des seules données de la TEMP cérébrale a permis de distinguer MP, PSP et DCB dans des groupes de patients cliniquement bien définis. Cette nouvelle approche statistique apporte des informations fiables. Cependant, une étude prospective consacrée à des syndromes parkinsoniens de novo serait utile pour préciser le potentiel de cette méthode.
The objective was to assess the value of single photon emission computerized tomography (SPECT) and factorial discriminant analysis (FDA) in the differential diagnosis of Parkinson's disease (PD), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD).
Sixty-two patients with clinical diagnoses of either CBD, PSP or PD were studied using brain HmPaO-SPECT. Thirteen pairs of regions of interest (ROIs) were drawn on the slices located 50 mm and 90 mm above the canthomeatal plane. Twenty-six uptake indices and 13 asymmetry indices were determined. FDA was performed in order to determine whether or not the patients could be classified into the correct clinical group on the basis of SPECT data alone. The most discriminant parameters were used to generate two predictive scores, which were tested in a second group of 15 patients.
FDA of all 39 variables correctly classified all the patients. A subset of 10 variables was used to build predictive scores, which correctly classified 90% of PD patients, 100% of PSP patients and 86% of CBD patients. When tested in the validation group of 15 patients, these predictive scores correctly classified 87% of the individuals. The frontal medial, temporoparietal and parietal regions were the most discriminant.
Using SPECT data alone, this study enabled us to distinguish between PD, PSP and CBD in patients with clear clinical presentations of the diseases in question. This novel, statistical approach provides reliable information. However, a prospective study dealing with de novo parkinsonian syndromes will be necessary.
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Levetiracetam reduces myoclonus in corticobasal degeneration: Report of two cases
2009, Journal of Neural Transmission
Corticobasal degeneration and Parkinson's disease asessed by HmPaO SPECT: The utility of factorial discriminant analysis
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View full text

Clinical studyClinical features and SPECT scanning in presumed cortico-basal ganglionic degeneration

Abstract

J Psychiatry Res

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Cortico-basal ganglionic degeneration with neuronal achromasia: clinical-pathological study of two cases

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Corticobasal degeneration: a unique pattern of regional cortical oxygen hypometabolism and striatal fluorodopa uptake demonstrated by positron emission tomography

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The metabolic landscape of corticobasal ganglionic degeneration: regional asymmetries studied with positron emission tomography

J Neurol Neurosurg Psychiatry

Clinical study
Clinical features and SPECT scanning in presumed cortico-basal ganglionic degeneration