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Year : 2000  |  Volume : 48  |  Issue : 4  |  Page : 405-6

Cortico-basal ganglionic degeneration--a frequently undetected syndrome.






How to cite this article:
Anand K S, Biswas A, Prasad A. Cortico-basal ganglionic degeneration--a frequently undetected syndrome. Neurol India 2000;48:405


How to cite this URL:
Anand K S, Biswas A, Prasad A. Cortico-basal ganglionic degeneration--a frequently undetected syndrome. Neurol India [serial online] 2000 [cited 2018 Dec 9];48:405. Available from: http://www.neurologyindia.com/text.asp?2000/48/4/405/1484




Cortico-basal ganglionic degeneration (CBGD) is a complex neurobehavioural disorder characterised by insidious onset and gradually progressive cerebrocortical and extrapyramidal dysfunction.
A 52 years old male presented in May 1998 with behavioural abnormalities since November 1996 and abnormal movements since June 1997. Initially the patient had difficulty in dressing himself. Soon afterwards he had difficulty in finding his way while inside the house. Sometimes he spent the whole night outside the house, to be discovered next morning by his family members in the nearby field. In April 1997 he also developed speech abnormality. His speech gradually became incomprehensible and he would laugh or cry without any reason. He also became forgetful. He developed twisting movements of right sided fingers, wrist and forearm and neck and lower limbs. He also developed parkinsonian features and was unable to perform activities of daily living on his own. There was no history of myoclonus, seizures, motor weakness, sensory or bladder and bowel symptoms.
On examination he was restless, laughing or crying without reasons. He could follow some verbal commands but could not do so when written commands were provided. He could neither utter a comprehensible word nor write a legible letter. His cranial nerves were normal. Examination of motor system was normal except the presence of akinesia, rigidity and dystonic posturing of limbs. These were more profound on the right side. There was no tremor or ataxia. His deep tendon reflexes as well as the plantars were normal. There was gross postural instability. The rigidity and akinesia did not respond to levodopa.
Investigations revealed normal haemogram, blood glucose, renal function and liver function tests. Serum calcium, phosphorous and alkaline phosphatase were 9.6 mg/dl,3.5 mg/dl and 11KAU/L, respectively. The serum copper was 90 mgm/dl and ceruloplasmin was 30 mg/dl. Serum VDRL was nonreactive and ELISA for HIV 1 and 2 were negative. CSF examination revealed a protein content of 20 mg/dl and sugar of 68 mg/dl and was acellular. The T2 weighted images on MRI [Figure. 1] showed prominent sylvian cisterns and prominence of cortical sulci particularly in frontal and both parietal lobes, suggesting symmetrical atrophy of frontal and parietal lobes. There was no KF ring on eye examination.
The typical features of CBGD can be categorised into movement disorders (akinesia, rigidity, postural instability, limb dystonia, cortical myoclonus and postural/intention tremor) and cortical signs, such as cortical sensory loss, apraxias and the 'alien limb' phenomenon.[1],[2] The most striking features of CBGD is asymmetry of involvement which differentiates it from most other neurodegenerative disorders. Rinne et al[2] reviewed 36 patients, with mean age at onset of 60.9+9.7 years. (range : 40-76 years). In the patients reported by Riley et al, the mean age at onset was 60 years (range: 51-71 years) and men were more commonly affected than women. Riley et al[1] reported apraxia in 71% of cases of CBGD. Although they found ideational and ideomotor apraxias to occur early and were sometimes the presenting symptoms, a variety of other apraxias have also been reported in CBGD. Alien limb phenomenon is an unusual sign in neurology and its presence, in the absence of a known callosal lesion, is highly suggestive of the diagnosis of CBGD. Speech abnormalities and aphasia has been reported to occur in 21% of patients with CBGD and are considered to reflect left hemisphere cortical pathology in this disorder.[1] Involvement of right parietal cortex in CBGD gives rise to visuospatial and constructional disturbances. Personality change, impaired attention, acalculia, impaired recall and learning, concrete thinking and left-right confusion have been noted in a number of patients. Oculomotility disturbances particularly manifested by impaired convergence and vertical and horizontal gaze palsy has been noted in CBGD. This feature sometime confuse CBGD with progressive supranuclear palsy (PSP). Dementia is a late feature of CBGD and was found in 43% of patients by Riley et al.[1] The full spectrum of clinical features typically seen in CBGD can also be present in patients with Pick's disease, but the latter disorder is usually dominated by cognitive, behavioural, and language disturbances such as primary progressive aphasia. Moreover, apraxia and parkinsonism, if present, are usually late finding in Pick's disease. Pathological features in CBGD include neuronal degeneration in pre-and post-central cortical areas, degeneration of basal ganglia, including substantia nigra (SN), and presence of achromatic neural inclusion seen not only in the cortex but also in the thalamus, subthalamus nucleus, red nucleus, and SN.[4] CT scans were abnormal in 14 of the 15 patients in one series, 8 had asymmetrical parietal lobe atrophy corresponding to the most affected side, and 6 had bilateral parietal atrophy.[1] PET scans shows reduced 18(F) fluorodopa uptake in the caudate and putamen, and markedly asymmetrical cortical hypometabolism in the superior temporal and inferior parietal lobe.[5]
Our patient presented with dressing and situational apraxias as the initial symptoms. Gradually, he developed abnormalities of speech, emotional lability, loss of social inhibition etc., the features of diffuse cortical dysfunction. These features were combined with asymmetric involvement of right side with extrapyramidal features like limb dystonia, akinesia, rigidity and postural instability. Our patients did not have 'alien limb' phenomenon which is a very interesting features of CBGD. However, its absence does not exclude the diagnosis, as it was observed only in 50% of patients by Riley et al.[1] The presence of features of cortical dysfunction in the early phase of illness compounded by extrapyramidal features and the absence of oculomotility disturbances and ataxia, clearly suggest the diagnosis of CBGD in our patients. These clinical findings were corroborated by symmetrical atrophy of frontal and parietal lobes in neuroimaging studies.

 

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1.Riley DE, Lang AE, Lewis A et al : Cortico-basal ganglionic degeneration. Neurology 1990; 40 : 1203-1212.   Back to cited text no. 1    
2.Rinne JO, Lee MS, Thompson PD et al : Cortico-basal degeneration: a clinical study of 36 cases. Brain 1994; 117 : 1183-1196.   Back to cited text no. 2    
3.Bogen JE : Split-brain syndrome. In: Handbook of clinical neurology. Vol. 1(45): Clinical Neuropsychology. Friederiks JAM, Ed. Amsterdam: Elselvier. 1985; 99-106.   Back to cited text no. 3    
4.Lippa CF, Cohen R, Smith TW et al : Primary progressive aphasia with focal neuronal achromasia. Neurology1991; 42 : 882-886.   Back to cited text no. 4    
5.Eidelberg D, Dhawan V, Moller JR et al : The metabolic landscape of cortico-basal ganglionic degeneration, regional asymmetries studies with positron emission tomography. J Neurol Neurosurg Psychiatry 1991; 54 : 856-862.   Back to cited text no. 5    

 

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Online since 20th March '04
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