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ORIGINAL ARTICLE
Year : 2013  |  Volume : 61  |  Issue : 1  |  Page : 7-11

Cerebral glucose metabolism, clinical, neuropsychological, and radiological profile in patients with corticobasal syndrome


Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Andhra Pradesh, India

Date of Submission12-Apr-2012
Date of Decision01-Jul-2012
Date of Acceptance08-Dec-2012
Date of Web Publication4-Mar-2013

Correspondence Address:
Surya Prabha Turaga
Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad 500 082, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.107916

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 » Abstract 

Background: Corticobasal syndrome (CBS) is characterized by progressive asymmetric rigidity and localized cortical disorders namely apraxia, cortical, sensory loss, focal dystonia, and refractory to levodopa treatment. Aim: To study the cerebral glucose-metabolism, cognitive, and magnetic resonance imaging (MRI) features in patients with CBS. Materials and Methods: Seventeen consecutive patients with CBS who fulfilled the criteria proposed by Lang et al., were studied. A detailed neurological including higher cortical functions and Unified Parkinson's Disease Rating Scale (UPDRS) on and off motor scores and neuropsychological assessment were done. All patients except one had undergone MRI brain and 18-Flouro 5-Deoxy Glucose Positron Emission Tomography (FDG PET CT) brain scan for assessing cortical atrophy and cerebral glucose metabolism, respectively. Results: Most of the 17 patients had Parkinsonian features with focal cortical signs of dystonia, apraxia, and cortical sensory loss and the hypokinesia was predominantly on left side. Neuropsychological assessment had shown impairment of frontal executive function, visuospatial function, and language. MRI brain showed asymmetrical cortical atrophy in left temparoparietal areas and basal ganglia. The MRI findings correlated with FDG PET brain scan findings, asymmetric focal hypometabolism in basal ganglia and inferior parietal and temporal, frontal lobes. Conclusion: 18 FDG PET brain is more sensitive than MRI brain in the early diagnosis of CBS and also correlates well with neuropsychological assessment.


Keywords: Apraxia, corticobasal syndrome, 5-Flouro 5-Deoxy Glucose Positron Emission Tomography, focal dystonia, neuropsychological tests


How to cite this article:
Turaga SP, Mridula R, Borgohain R. Cerebral glucose metabolism, clinical, neuropsychological, and radiological profile in patients with corticobasal syndrome. Neurol India 2013;61:7-11

How to cite this URL:
Turaga SP, Mridula R, Borgohain R. Cerebral glucose metabolism, clinical, neuropsychological, and radiological profile in patients with corticobasal syndrome. Neurol India [serial online] 2013 [cited 2019 Sep 18];61:7-11. Available from: http://www.neurologyindia.com/text.asp?2013/61/1/7/107916



 » Introduction Top


Corticobasal syndrome (CBS) is characterized by progressive asymmetric rigidity/dystonia and localized cortical signs. [1],[2],[3] The pathologic hallmark of CBS is the presence of swollen, achromatic neurons, and asymmetric cortical atrophy, which is most pronounced in posterior frontal and parietal lobe. [4] The motor symptoms of CBS are refractory to levodopa and dopamine agonists. Because of asymmetric nature of the disease, sometimes CBS can be mistaken for Parkinson's disease, however, the prognosis is different. Although there are clinical diagnostic criteria for CBS, there is a lot of overlap with other akinetic disorders and a misdiagnosis of progressive supranuclear palsy can be done in 40% of cases. [5] Recent clinical studies emphasize specific patterns of cognitive impairment with involvement of visuospatial, language, and praxis. CBS can initially manifest as cognitive disorder and distinctive neuroradiological findings can assist the clinical diagnosis. [6] Conventional imaging methods such as magnetic resonance imaging (MRI) brain often may not reveal any abnormality. [7] Positron emission tomography (PET) brain may be employed as an additional test to clinical diagnosis in equivocal cases. [8] In CBS, there is asymmetric hypometabolism of the striatum, thalamus, frontal and temparoparietal cortices, with the hemisphere contralateral to the most affected limb displaying the greatest reduction. This study is aimed at studying cerebral glucose metabolism, cognitive and MRI features of consecutive patients with CBS presenting to movement disorders clinic in a tertiary care centre in south India.


 » Materials and Methods Top


This study included 17 consecutive patients with CBS presenting to movement disorders clinic. Nizam's Institute of Medical Institute who fulfilled the criteria of Lang et al. [1] Inclusion criteria included: Asymmetric rigidity/dystonia and focal reflex myoclonus plus one cortical sign (apraxia, cortical sensory loss, or alien limb).Exclusion criteria included: Early dementia; early vertical gaze palsy; rest tremor; severe autonomic disturbances; sustained responsiveness to levodopa; lesions on imaging studies indicating another pathologic condition.

All the patients had detailed neurological assessment, including higher mental examination and Unified Parkinson's Disease Rating Scale (UPDRS) on and off motor score after taking written consent and also performed the Ideomotor Apraxia test with both arms. [9],[10] They also had detailed neuropsychological assessments for lobe functions. [11-13] Mini Mental State Examination (MMSE) [14] and Addenbrooke's Cognitive Examination-revised (ACE-R) [15],[16] to assess general cognitive status. We selected tests that are widely used in routine neuropsychological practice and validated in Indian context and are sensitive to detect early deficits in cognitive domains. Executive functions were assessed by tests like phonemic fluency and animal fluency test, verbal working memory, attention by digit span, planning by Tower of London, response inhibition by Stroop test. Naming was assessed using 12 line diagrams taken from the ACE-R. Visual and verbal memory, visuospatial orientation were assessed by complex figure test, Rey's verbal memory learning test, block design test respectively. Comparison was done with age and education matched normal controls. Values below 2 SD from the mean are considered abnormal.

All patients except one had brain magnetic resonance imaging (MRI) with 1.5 Tesla machine and 5-Flouro 5-Deoxy Glucose Positron Emission Tomography (FDG PET CT) brain scan for assessing cortical atrophy and cerebral glucose metabolism respectively. For FDG PET CT brain scan, patients are instructed to fast and asked not to consume beverages, except for water, for at least 4-6 hours before the administration of 18 F-FDG to decrease physiologic glucose levels and to reduce serum insulin levels to near basal levels. Oral hydration with water is encouraged. Intravenous dextrose containing fluids or parenteral feeds also withheld for 4-6 h. The patient were kept in a quiet and dimly lit room for 18 F-FDG administration and the subsequent uptake phase. This FDG PET is a semi-qualitative method to assess the glucose metabolism. MRI brain and PET-CT brain were read by 2 radiologists who are blinded to each other's report.

Statistical analysis

The sensitivity of MRI and FDG-PET was calculated in comparison to clinical criteria and chi square test was used to analyze difference between two tests and the correlation between clinical features and imaging.


 » Results Top


Of the 17 patients, 11 were men and all were right handed. Education status was 13 college education and 4 school education. Mean age at presentation was 62.29 ± 7.28 years and mean duration of disease was 4.06 ± 2.28 years. Mean Hohen and Yahr score was 3.23 ± 1.2 and mean UPDRS motor off score was 46.70 ± 12.59 and on score was 41.06 ± 13.8.

All the 17 patients fulfilled the diagnostic criteria for CBS proposed by Lang et al. [1] All patients with CBS had an asymmetrical bradykinetic rigid syndrome. Left side was predominantly involved in 13 (76.5%) patients and right side in 4 (23.5%) patients [Table 1]. Performance on neuropsychological tests of the patients was compared with that of normal control subjects matched for age and education [Table 2]. There was no difference in P value quantitatively in tests of episodic memory category, fluency, and naming. Comparison of cognitive impairment among patients with right or left predominant hypokinesis showed no statistical difference in the cognitive profile with a trend toward significance in language impairment predominantly in patients with right hypokinesia patients and visuospatial impairment in predominantly left hypokinetic patients.
Table 1: Demographic data of corticobasal syndrome patients


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Table 2: Neuropsychological assessment among patients with corticobasal syndrome and controls


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MRI was contra-indicated in one patient as he had metal implants, for him computed tomography (CT) scan brain was done. MRI showed asymmetrical cortical atrophy in left temparoparietal areas and basal ganglionic atrophy in 75% with positive correlation with clinical diagnosis [Figure 1]. Associated frontal lobe atrophy was noted in four patients [Table 3]. FDG PET CT brain scan was done in all patients and 15 patients showed asymmetric focal hypometabolism in basal ganglia and inferior parietal and temporal, frontal lobes [Figure 2], which correlated with the MRI brain and the clinical findings of asymmetric rigidity, dystonia, apraxia (88.3%). Frontal lobe involvement especially dorsolateral, medial prefrontal lobe hypometabolism was seen in 13 (76%) patients [Table 3]. FDG PET showed a higher correlation (88.9%) with the clinical diagnosis of CBS in comparison to MRI (75%).There was a significant difference in picking up frontal lobe dysfunction with hypometabolism seen in 75% compared with 25% atrophy on MRI. Concordance with clinical evidence of frontal executive dysfunction was seen in 28.75% on MRI compared with significantly higher percentage (85.71%) on FDG PET ( P =0.003). On correlating with other cognitive impairments, right sided temparoparietal involvement was seen in 90% of patients with visuospatial disturbances and left temparoparietal involvement was noted in 100% of patients with language impairment on both MRI and 18 FDG PET [Table 4].
Table 3: MRI findings (n = 16) and flouro deoxy glucose positron emission tomography in corticobasal syndrome (n = 17)


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Table 4: MRI findings and flouro deoxy glucose positron emission tomography in corticobasal syndrome in correlation to neuropsychological tests


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Figure 1: MRI Brain axial section T1-weighted image showing asymmetric atrophy of left temparoparietal area

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Figure 2: FDG PET Brain scan showing hypometabolism of left frontal, temparoparietal area

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 » Discussion Top


This study confirms that CBS patients show a characteristic asymmetric pattern of impaired glucose metabolism affecting the thalamus, lenticular nucleus and selective cortical regions, inferior parietal, sensorimotor, lateral temporal, and medial and lateral frontal. While the dorsolateral frontal and medial frontal cortices are affected in CBS, the orbitofrontal cortex seems to be spared distinguishing this condition from frontal dementias. The occipital cortex and the superior parietal cortex are also found to be relatively unaffected thus correlating with the selective cognitive impairment seen in CBS. The presence of asymmetric cerebral hypometabolism in patients with CBS is consistent with previous single photon-emission computed tomography (SPECT) [17] and PET investigations. Asymmetric pattern of impaired glucose metabolism in temparoparietal cortex contralateral to the most affected limb was seen in 88.3% of our patients with CBS with involvement of medial and lateral frontal cortex in 75%. This is consistent with previous studies with 18 FDG PET in CBS. [8],[18] The reduced glucose metabolism in the corpus striatum may be due to direct pathologic involvement or loss of cortical input. It is less likely to be due to damage of the nigrostriatal dopaminergic system in CBS, because this occurs in Parkinson's disease without any reduction in striatal metabolism.

On comparison of MRI and FDG PET in CBS, in our study the imageological changes in these patients were mild and did not encompass the entire area of cortical hypometabolism noted on the PET scans. There was a significant difference in demonstrating frontal lobe impairment by FDG PET than MRI, correlating better with clinical features. Thus the extent of involvement of the brain in CBS is better delineated by 18 FDG PET. [18]

The mean age of presentation is similar to other studies. Mean duration of disease was higher in our study and it may be due to advanced status of the disease in our patients. [5] In our study we found predominant left sided akinesis in three-fourth of the patients. Over representation of left sided symptoms have been described in previous studies also. [4],[5] However, a recent study showed predominance of right sided motor symptoms and left hemispheric involvement in patients with cognitive onset of CBS, especially with language impairment. [19]

In our study, majority of the patients with CBS done poorly in tests for frontal executive function including tests for phonemic fluencies, planning and response inhibition. Similar results are described in the earlier studies with significant frontal executive dysfunction and visuospatial impairment; however, language impairment is also seen in a larger proportion. [20] In our study, category (animal) fluency and naming is relatively spared suggesting involvement of language impairment being mild and phonemic fluency impairment may also be partly due to frontal executive dysfunction. [20] The predominant involvement of right hemisphere in most of our patients may explain the lack of significant language impairment. [19] This may be secondary to small number of predominant right hypokinesia patients and more widespread disease among predominant right hypokinesia patients with language impairment. A recent study has correlated the cognitive deficits with autopsy findings with maximum pathological changes in medial and lateral frontal cortex, basal ganglia with intermediate severity of involvement in parietal cortex and least involvement of medial temporal cortex. [20]

In our study, MRI brain findings of asymmetric parietal atrophy is similar to the observations by Soliveri et al. [6] This correlated well with clinical diagnosis of CBS. Although MRI revealed asymmetric brain atrophy in CBS patients, the radiographic changes in these patients were mild and would not fully account for the cortical hypometabolic changes noted on the PET scans. It is most likely, therefore, that the significant decline in hypometabolism in CBS is a reflection of the extensive and regionally selective cortical damage documented in necropsy studies. [5]

In conclusion neuropsychological assessment showed impairment of frontal executive function, visuospatial function and phonemic fluencies in patients with CBS. MRI brain and 18 FDG PET were concordant with the clinical diagnosis of CBS in 75% and 88%, respectively, with asymmetric temporoparietal atrophy on MRI and asymmetrical frontoparietal hypometabaolism on PET scans. Concordance with frontal executive dysfunction was significantly more with 18 FDG PET when compared with MRI brain. True extent of involvement in patients with CBD is better delineated by 18 FDG PET and correlated better with the cognitive impairment and so aid in early detection CBS.

 
 » References Top

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    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]

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