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CASE REPORT
Year : 2013  |  Volume : 3  |  Issue : 2  |  Page : 156-159

Dementia with Lewy bodies: Enigmatic presentation


1 Psychiatry Residency Program, Oman Medical Specialty Board, Oman
2 Department of Behavioral Medicine, Sultan Qaboos University Hospital, Oman

Date of Submission14-Aug-2012
Date of Acceptance05-Sep-2012
Date of Web Publication3-Jun-2013

Correspondence Address:
Ahmed Al-Harrasi
Department of Behavioral Medicine, Sultan Qaboos University Hospital, P.O. Box 38, Al-Khod
Oman
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2231-0738.112847

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   Abstract 

Illness may present clinically in different ways causing misdiagnosis of the case and inappropriate treatment; an example of this is dementia with Lewy bodies. This neurodegenerative disorder has overlapping features with both Alzheimer's disease and Parkinson's disease dementia, which tends to confuse the clinician to arrive at the right diagnosis. It is important that clinicians update their knowledge of the diagnostic criteria of dementia with Lewy bodies in order to provide suitable pharmacological and non-pharmacological management for its cognitive, neuropsychiatric, motor and sleep disturbances without causing distressing side effects by inappropriate drug prescription. This article will describe a case of dementia with Lewy bodies with literature review.

Keywords: Alzheimer′s disease, dementia with Lewy bodies, Parkinson′s disease dementia, Parkinson′s disease psychosis, Parkinson′s disease


How to cite this article:
Al-Harrasi A, Aravazhi M S, Al-Sinawi H. Dementia with Lewy bodies: Enigmatic presentation. Int J Nutr Pharmacol Neurol Dis 2013;3:156-9

How to cite this URL:
Al-Harrasi A, Aravazhi M S, Al-Sinawi H. Dementia with Lewy bodies: Enigmatic presentation. Int J Nutr Pharmacol Neurol Dis [serial online] 2013 [cited 2020 Jun 6];3:156-9. Available from: http://www.ijnpnd.com/text.asp?2013/3/2/156/112847


   Background Top


Dementia with Lewy bodies (DLB) is considered the second most common cause of dementia in the elderly after Alzheimer's disease (AD). [1] DLB is a neurodegenerative disorder that progresses gradually and is characterized by the core features of cognitive impairment, psychosis and  Parkinsonism More Details. [1],[2],[3] The Lewy body proteins are intraneuronal cytoplasmic inclusions found diffusely throughout the cortical and subcortical areas of the brain. [1]

DLB has overlapping characteristics with AD and Parkinson's disease dementia (PDD), which may increase the chances of misdiagnosis. [4],[5] Hence, it is essential to differentiate between DLB, AD and PDD to provide separate and appropriate treatment strategies. The confusion between DLB and PDD is the occurrence of parkinsonian symptoms as a core feature in DLB. [6] DLB can also be confused with Parkinson's disease psychosis (PDP). In this condition psychotic symptoms develop in a patient with progressive Parkinson's disease (PD). [7] However, careful evaluation of the symptoms would lead to a more reliable diagnosis of DLB. Below we describe a case which had challenged us to reach a reliable diagnosis because of its ambiguous presentation and comorbidities.

Presentation

An 83-year-old man was referred to our psychiatry clinic by a neurologist in August 2011 with visual hallucinations and cognitive decline over the past 3 months. His wife reported that he had difficulty initiating and maintaining sleep throughout the night. He also had poor appetite, confusion and irritability. The confusions were worse in the late evening and at night. He had been having tremors in both hands for 50 years. No other tremor was known involving the head or legs. This type of tremor was a frequent occurrence in his family. He also had slow gait and unchanged facial expression.

Medical history

Medical problems included diabetes, hypertension, recurrent syncopal attacks and hypochromic microcytic anemia for which he was on multiple medications. He was seen by a neurologist for evaluation of his symptoms and was started on Sinemet and Pramipexole. Gradually, he developed sleep disturbances and visual hallucinations.

Personal and family history

He had an uneventful childhood and attended school in Oman; then, worked in the government. He got married at the age of 28 and has 5 children. He retired from the job at the age of 60. His daughter died a few months back due to cancer. He has no past history of psychiatric illness. There was no family history of dementia or PD.

Mental state examination

He was well dressed, calm and cooperative with good eye contact. His speech was relevant and coherent and he appeared to have euthymic mood. He did not report any abnormal thought content. He described seeing children around when no one was there in the ward. He was disoriented to time, place and person and could not recognize even his wife. The mini-mental state examination (MMSE) was assessed to be 12/30.

Physical examination

During the examination, the patient was conscious but disoriented. There was axial tremor. The examination for cranial nerves revealed distinctly restricted visual acuity in the right eye. The left eye had cataract hence, the examination of visual field was restricted. Glabellar reflex was found to be non-habituated, and a positive palmo-mental reflex could be demonstrated. Other cranial nerves were found to be intact. Regarding the motor function, there was no paresis in any limb. The leg holding test was inconspicuous. The proprioceptive reflexes were more on the left arm compared to the right. In the legs the reflex was symmetric and moderately exaggerated. Babinski's sign was negative. The coordination tests showed intact finger-nose test, the left side showed dysdiadochokinesia and there was a distinct decrement in finger tapping. The knee-heel test was slowed down, but not atactic. There was a left focus holding, resting tremor, and left sided rigidity on the arms, but no severe postural instability. On gait testing the left arm did not swing; there was small stepped gait, increased turn step number, slight camptocormia, and a marked tremor of the left leg. There was hyperesthesia in the left C5 region.

Investigations

Blood work up was unremarkable apart from hypochromic microcytic anemia. The magnetic resonance imaging (MRI) of the brain showed generalized brain atrophy with widening of the ventricles and old ischemic changes [Figure 1] and [Figure 2]. The Extra Cranial Duplex Sonography showed right focus moderate arterio-sclerotic macro-angiography in carotids with echo-rich plaques. Electroelncephalogrphy (EEG) showed no specific changes. Nerve conduction and electromyographic (EMG) study indicated demyelinating polyneuropathy.
Figure 1: MRI images showing cortical brain atrophy with widening of the ventricles and sulci. (a) T1, axial. (b) T2, axial

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Figure 2: MRI image showing an old cerebellar infarct (circle) as part of generalized ischemic changes (T2, axial)

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Differential diagnosis

Dementia with Lewy bodies, Parkinson's disease dementia, Parkinson's disease psychosis.

Progress of the case

During the course of his admission in the hospital, blood tests were done to rule out any reversible cause of dementia. Clonazepam 500 mcg and Mirtazapine 7.5 mg at night were added for one week; the dose of Mirtazapine was then increased to 15 mg. Quetiapine was also started at 25 mg BID. Pramipexole and Clonazepam were later discontinued due to worsening psychiatric condition. He became less irritable and less aggressive, but his hallucinations and confusion became more prominent. The family asked for discharge in order to take him to Germany for a second opinion.

In Germany, he was examined by a panel of neurologists and psychiatrists. The diagnosis of PD/PDD was considered to be less apparent, since he did not improve with the PD medications; rather, the psychotic symptoms were getting worse. The confusion was relapsing in early evenings; he did not recognize his family and constantly wanted to leave the hospital. A battery of neuropsychological tests was done which showed substantial cognitive impairment. The conclusion was that DLB was the most likely diagnosis, L-Dopa medications were prescribed under which the Parkinson's symptoms improved to a certain extent, but there was still recurring confusion. Therefore, after a period of tapering, L-Dopa therapy was stopped. Quetiapine was increased to 25 mg QID and transdermal Rivastigmine was started.

Outcome

Few days after reviewing his medications he showed good improvement on Rivastigmine therapy. His sleep and mobility improved and he had fewer episodes of confusion. His aggressiveness and irritability decreased in severity. The levels of vitamin B12 and folic acid were normal. So, most likely the polyneuropathy was a complication of diabetes mellitus; with the supporting fact of increased HbA1c indicating poor glycemic control. Metformin was started on which the daily blood sugar profile improved. Because of recurrent syncope, trans-esophageal echocardiogram (TEE) was done and indicated diastolic dysfunction with good left ventricular function. Cardiac check-up was recommended. Iron tablets were started for the hypochromic microcytic anemia.


   Discussion Top


This case depicts many important diagnostic issues as well as therapeutic strategies in DLB. This patient initially had tremors which were familial. The tremors were assumed to be part of PD and were treated with anti-Parkinson's drugs, which exacerbated the cognitive impairment and psychiatric symptoms. Since the other two core features (visual hallucinations and fluctuating cognitive impairment) required to substantiate the diagnosis of DLB were not strongly pronounced in the early stages of the disease in this case, the condition was confused with PDP. Hence, the accurate diagnosis could not be reached at the initial stage.

The diagnosis of DLB can be made by analyzing the features the patient presents with. According to the consensus criteria for clinical diagnosis, the presence of any two of the core features (fluctuating cognition, recurrent visual hallucination and spontaneous motor features of parkinsonism) are essential for a diagnosis of probable DLB and one is essential for possible DLB. [2],[3],[8] This patient had all the three core features and also progressive dementia which is a central feature of DLB. Moreover, other suggestive features like rapid eye movement (REM) sleep behavior disorder, syncope, delusions, aggression, depression and autonomic dysfunction were also present to support the diagnosis. Since the motor symptoms were prominent, the diagnosis was a challenge.

This case has to be differentiated from PD/PDD which usually presents with rigidity, bradykinesia, gait abnormality, mask-like faces, reduced arm swing and tendency to fall. [6] Resting tremor is more predominant in PD/PDD than in DLB. [9] However, the occurrence of dementia within a year of extrapyramidal symptoms favors the diagnosis of DLB. [3] Medications used to treat PD may contribute to PD psychosis, but the timing of the onset of the psychotic symptoms is important to decide whether it is DLB or PDP. As in this case, if the hallucinations become apparent shortly after the initiation of the dopaminergic medications, DLB should be considered. [7]

With regards to treatment, cholinergic defects have been found to be more pronounced in DLB compared to AD; [10] hence, adding acetylcholinesterase (Ach-E) inhibitors has a potential effect on cognitive function in DLB. There is evidence from double-blinded, placebo-controlled studies that Rivastigmine alleviates neuropsychiatric symptoms associated with DLB. [11] In this patient the addition of transdermal Rivastigmine patch showed a great improvement in cognitive functions, as well as in the severity of apathy, hallucinations and delusions. Patients with DLB show less improvement with L-Dopa/carbidopa combination as compared to patients who have PD/PDD. [12],[13],[14] The probability that L-Dopa preparations would exacerbate psychotic symptoms in patients with DLB is high. [14] As in this case, aggression and confusion were aggravated when the patient was on L-Dopa medications; whereas, his psychotic symptoms reduced when L-Dopa was stopped. This can be a strong fact to differentiate this case of DLB from PDD or PDP. Therefore, the treatment of extrapyramidal symptoms should be aimed at improving the patient's mobility without worsening the psychosis. Atypical anti-psychotics are better tolerated in DLB for the management of psychotic symptoms. [15] In this case the dosage of quetiapine was gradually titrated and increased which showed better results.

Research has been ongoing to find new methods of treating dementia and managing its associated symptoms. Most of the recent studies on newer, proposed treatment methods focused on AD and PD; however, because of the similarities between DLB and these diseases we can use those treatments to study them on patients with DLB and its associated symptoms. Guest and Grant discussed the role of various oxidative stress mechanisms in the development of neurodegenerative diseases; and stressed on the fact that neurons lack the antioxidant, protective processes found in other cell types. They recommended the use of dietary supplements of vitamin E, ascorbate, carotenoids and plant phenols to foster antioxidant neuro-protection and prevent further neurodegenerative damage. [16] Another group of researchers reported a neuroprotective role for gamma-tochopherol in mice with PD. It was found to improve different motor symptoms through removal of free radicals and anti-inflammatory role. [17] Furthermore, Singhal et al., reviewed evidence of using some herbs in treating patients with AD and ameliorating associated symptoms. They found stronger evidence for formulae containing salvia, gingko and huperzine A. [18] Although these treatment methods can be tried in patients with DLB, they require further research to establish their effectiveness and safety in such patients.


   Conclusion Top


The inference from the this case is that it is highly essential to carefully evaluate the symptoms and diagnose DLB at the earliest for effective management and prevent further complications. It is of prime importance to differentiate DLB from AD, PD/PDD and PDP to provide appropriate pharmacological and non-pharmacological treatment. The more insights that can be gained from these attempts to help severely ill patients will hopefully generalize to more effective treatment for other dementia sufferers.

 
   References Top

1.McKeith I, Mintzer J, Aarsland D, Burn D, Chiu H, Cohen-Mansfield J, et al. Dementia with Lewy bodies. Lancet Neurol 2004;3:19-28.  Back to cited text no. 1
    
2.McKeith IG, Dickson DW, Lowe J, Emre M, O'Brien JT, Feldman H, et al. Diagnosis and management of dementia with Lewy bodies: Third report of the DLB Consortium. Neurology 2005;65:1863-72.  Back to cited text no. 2
    
3.McKeith IG, Galasko D, Kosaka K, Perry EK, Dickson DW, Hansen LA, et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 1996;47:1113-24.  Back to cited text no. 3
    
4.Byrne EJ. Diffuse Lewy body disease: Disease spectrum disorder or variety of Alzheimer's disease. Int J Geriatr Psychiatry 1992;7:229-34.  Back to cited text no. 4
    
5.Gnanalingham KK, Byrne EJ, Thornton A, Sambrook MA, Bannister P. Motor and cognitive function in Lewy body dementia: Comparison with Alzheimer's and Parkinson's diseases. J Neurol Neurosurg Psychiatry 1997;62:243-52.  Back to cited text no. 5
    
6.Aarsland D, Ballard C, McKeith I, Perry RH, Larsen JP. Comparison of extrapyramidal signs in dementia with Lewy bodies and Parkinson's disease. J Neuropsychiatry Clin Neurosci 2001;13:374-9.  Back to cited text no. 6
    
7.Metelerkamp B, Tang D. Psychosis in Parkinson's disease. Geriatr Med 2010;40:686-91.  Back to cited text no. 7
    
8.McKeith IG, Perry EK, Perry RH. Report of the second dementia with Lewy body international workshop: Diagnosis and treatment. Consortium on Dementia with Lewy Bodies. Neurology 1999;53:902-5.  Back to cited text no. 8
    
9.Latoo J, Jan F. Dementia with Lewy bodies: Clinical review. Br J Med Pract 2008;1:10-4.  Back to cited text no. 9
    
10.Gomez-Tortosa E, Ingraham AO, Irizarry MC, Hyman BT. Dementia with Lewy bodies. J Am Geriatr Soc 1998;46:1449-58.  Back to cited text no. 10
    
11.Wesnes KA, McKeith IG, Ferrara R, Emre M, Del Ser T, Spano PF, et al. Effects of rivastigmine on cognitive function in dementia with Lewy bodies: A randomised placebo-controlled international study using the cognitive drug research computerised assessment system. Dement Geriatr Cogn Disord 2002;13:183-92.  Back to cited text no. 11
    
12.Molloy S, McKeith IG, O'Brien JT, Burn DJ. The role of levodopa in the management of dementia with Lewy bodies. J Neurol Neurosurg Psychiatry 2005;76:1200-3.  Back to cited text no. 12
    
13.Lebert F, Le Rhun E. Treatment of dementia with Lewy bodies. Rev Neurol (Paris) 2006;162:131-6.  Back to cited text no. 13
    
14.Goldman JG, Goetz CG, Brandabur M, Sanfilippo M, Stebbins GT. Effects of dopaminergic medications on psychosis and motor function in dementia with Lewy bodies. Mov Disord 2008;23:2248-50.  Back to cited text no. 14
    
15.Fernandez HH, Trieschmann ME, Burke MA, Friedman JH. Quetiapine for psychosis in Parkinson's disease versus dementia with Lewy bodies. J Clin Psychiatry 2002;63:513-5.  Back to cited text no. 15
    
16.Guest JA, Grant RS. Effects of dietary derived antioxidants on the central nervous system. Int J Nutr Pharmacol Neurol Dis 2012;2:185-97.  Back to cited text no. 16
  Medknow Journal  
17.Karunanithi K, Annadurai A, Krishnamoorthy M, Elumalai P, Manivasagam T. 1-methyl 4-phenyl 1,2,3,6-tetrahydropyridine is a potent neurotoxin: Gamma-tocopherol recuperate behavior, dopamine, and oxidative stress on parkinsonic mice. Int J Nutr Pharmacol Neurol Dis 2011;1:139-45.  Back to cited text no. 17
    
18.Singhal AK, Naithani V, Bangar OP. Medicinal plants with a potential to treat Alzheimer and associated symptoms. Int J Nutr Pharmacol Neurol Dis 2012;2:84-91.  Back to cited text no. 18
  Medknow Journal  


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