|Year : 2015 | Volume
| Issue : 1 | Page : 57-59
Corticobasal degeneration presented as rapidly progressive dementia
Sunil Suthar, Ravi M Soni, Isha Sharma, Sarvada C Tiwari
Department of Geriatric Mental Health, King George's Medical University, Lucknow, Uttar Pradesh, India
|Date of Web Publication||23-Jul-2015|
Dr. Sunil Suthar
Department of Geriatric Mental Health, King George's Medical University, Lucknow, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
Corticobasal degeneration (CBD) rarely presents with rapidly progressive dementia (RPD). Its description primarily exists as case reports and case series only. This case report describes and discusses RPD as an initial manifestation of CBD for the benefit of clinician. A 75-year-old male who presented initially with complaints of difficulty in money handling, repetitive activities, irritability, and naming difficulty for 8 months. Later, he developed complaints of forgetfulness, navigational difficulty, dystonia, and rigidity in the right hand. This case demonstrates that patients who are presenting with RPD in geriatric mental health, motor symptoms must be evaluated to rule out corticobasal degeneration.
Keywords: Corticobasal degeneration, geriatric mental health, rapidly progressive dementia
|How to cite this article:|
Suthar S, Soni RM, Sharma I, Tiwari SC. Corticobasal degeneration presented as rapidly progressive dementia. J Geriatr Ment Health 2015;2:57-9
|How to cite this URL:|
Suthar S, Soni RM, Sharma I, Tiwari SC. Corticobasal degeneration presented as rapidly progressive dementia. J Geriatr Ment Health [serial online] 2015 [cited 2019 Dec 15];2:57-9. Available from: http://www.jgmh.org/text.asp?2015/2/1/57/161387
| Introduction|| |
In contrast to most of degenerative dementias, which have insidious onset with gradual progression over years, the rapidly progressive dementias (RPDs) can have a subacute onset from days to month, and fatal outcome.  Hence, it is important to recognize RPDs at the earliest. Corticobasal degeneration (CBD) is a rare gradually progressive neurodegenerative disorder that may present as RPD. It was first described by Rebeiz et al. in 1968, and diagnostic criteria for probable CBD were laid by Armstrong et al.  As per this criteria CBD presents with history of insidious onset, gradually progressive dementia, within duration of 1-year, with age at onset after 50 years, with no family history and no genetic mutation that affects tau proteins. It is characterized by the presence of asymmetric presentation of any two of the following symptoms namely limb rigidity or akinesia, limb dystonia, limb myoclonus along with any two of the symptoms namely, oro-buccal or limb apraxia, cortical sensory deficit, alien limb phenomenon. Both genders are equally affected. Higher cortical symptoms affected in CBD include cognitive impairment, aphasia, limb apraxia, cortical sensory loss, and alien limb phenomenon. Psychological symptoms include behavioral changes and depression.  CBD may present to neurologist as movement disorder or to psychiatrist for cognitive and behavioral symptoms. In this case, CBD presented as a case of rapidly progressive frontotemporal dementia in a psychiatric setup. Cognitive and behavioral symptoms have been reported as early symptoms in 21% cases of pathological diagnosed CBD,  but this case is unique as there is no report of CBD presenting as an RPD till date.
| Case Report|| |
A 75-year-old male, retired bank cashier presented with complaints of difficulty in money handling, repetitive activities (checking and closing the door), irritability, naming difficulty, decreased sleep and appetite for 8 months. There was no history of recreational drug use and promiscuity. The family and past psychiatric history was unremarkable. On the basis of clinical history and the examination provisional diagnosis of frontotemporal dementia was considered. Tablet sertraline was prescribed for irritability and repetitive behavior. After about 4 months, there was an improvement in irritability and repetitive activities but over the next few months patient developed recent memory loss, navigational difficulty, along with dystonia and rigidity in the right hand. The patient was admitted for detailed evaluation and further management. He scored 18 on Hindi mental status examination (HMSE).  Routine biochemical parameters (including Vitamin B 12 and thyroid) were within normal limits. Magnetic resonance imaging (MRI) findings showed diffuse cerebral atrophy (predominantly left side) with ischemic demyelination. On the basis of diagnostic criteria for CBD developed by Armstrong et al.  neurologist diagnosed the patient as suffering from probable CBD. An adequate trial of oral levodopa was given for dystonia, rigidity, and bradykinesia in the right hand, but there was no improvement. The patient was prescribed physiotherapy for further management during follow-up. As there are no specifically recommended drugs for managing cognitive symptoms in frontotemporal dementia,  as well as for CBD. The trial of tablet donepezil up to 10 mg was given for 2 months with no improvement in HMSE score.
| Discussion|| |
Dementia is one of the major psychiatric causes of morbidity and disability among elderly. Rapidly progressive presentation of dementia is not a common presentation. It exists only in the form of case reports and case series in the literature. Amongst RPDs, CBD presenting as dementia is still rarer. . The overall prognosis of dementia is poor in such a setting. Only in cases of reversible dementias where etiology is known there are chances for recovery. In other cases also if the underlying cause is managed, there are fair chances of retarding the progression of dementia. CBD can be managed with neurological interventions.
The presented case had very characteristic features of RPD. The definition of RPD is quite variable. University of California, San Francisco group defines RPD as a form of dementia in which fully formed dementia syndrome emerges within 2 years of appearance of the initial symptoms. Another group defines RPD as rapid cognitive decline that occurs in an individual who already has an established diagnosis of dementia, but which is declining at a rate not consistent with the usual course of the illness. In fourth, Canadian consensus conference on the diagnosis and treatment of dementia, Moore et al.  suggested diagnostic criteria for RPDs - (a) It develops within 12 months after the appearance of first cognitive symptom, (b) exclusion of delirium, (c) for Alzheimer dementia decline of three or more points on MMSE in 6 months. The rapidly progressive cognitive decline occurred within 8 months in our case and meets the diagnostic criteria of RPD. Due to predominant behavioral and other frontal lobe signs a subcategory of frontotemporal dementia was provisionally entertained. The fine neurological mechanism became evident later on, with the appearance of neurological symptoms that a possibility of CBD with frontotemporal dementia could be considered. CBD often resembles and generally misdiagnosed as Creutzfeldt-Jakob disease More Details (CJD). Though rare in our setup, CJD was ruled out as MRI and electroencephalogram did not reveal characteristic changes. As evident from the literature there is substantial overlap between frontotemporal dementia and CBD, both of which are tauopathies.  Hence, the co-occurrence of both conditions should be considered together whenever there is a clinical presentation marked by rapid cognitive decline with atypical extrapyramidal symptoms. Although CBD is an uncommon cause of RPD but while evaluating a patient with RPD, the presence of motor symptoms for early diagnosis of CBD must be looked for.
The authors are thankful to Prof. Hitesh Khurana, Department of Psychiatry, Pt BD Sharma PGIMS, Rohtak for his critical and constructive comments for finalizing this manuscript.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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