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Letter   |    
Rapidly Progressive Dementia and Myoclonus
Emmanuel Sagui, M.D.; Michel Bregigeon, M.D.; Christian Brosset, M.D.; Celine Tilignac, M.D.; Jean Louis Kemeny, M.D.; Pierre Clavelou, M.D.; Jean Francois Pellissier, M.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 2000;12:412-412. doi:10.1176/appi.neuropsych.12.3.412
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Dementia With Lewy BodiesMyoclonus

SIR: Dementia with Lewy bodies (DLB) is a lately identified concept based on a clinical and pathological correlation: progressive disabling mental impairment with peculiar features such as hallucinations, fluctuating cognition or motor parkinsonism, and cortical with or without subcortical Lewy bodies.1 We report briefly two cases with unusual features of DLB.

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Case Reports

Case 1. A 74-year-old woman with no significant medical history but with depression 1 year ago began complaining of slowness of movements. Examination disclosed mild features of parkinsonism, including reduced armswing, rigidity, and bradykinesia, without resting tremor. Mini-Mental State Examination (MMSE) score was 30/30. Shortly after low doses of levodopa (150 mg) were given, parkinsonian features receded, but visual complex hallucinations arose. These hallucinations were not recognized as unreal and even worsened after levodopa was stopped or clozapine was given. Cognitive impairment appeared 7 months later. Hospitalization then occurred as myoclonus became evident. MMSE was 6/30, with perseveration and rigid-akinetic signs at examination. Decline was markedly rapid, and the patient died 8 months after the onset of the symptoms. Brain computed tomography (CT) revealed cerebral atrophy. EEG showed triphasic complexes without periodicity. 14-3-3 protein was not detected in the cerebrospinal fluid (CSF). Diffuse cortical Lewy bodies were found at autopsy.

Case 2. A 72-year-old man with history of tobacco addiction and asbestosis presented with a 1-year history of progressive forgetfulness. Examination disclosed mild temporospatial confusion, dressing and ideomotor apraxia, and bilateral cogwheel rigidity. Language and attention were well preserved. Brain CT was normal. Six months later, the patient came to the hospital for cognitive impairment with shuffling gait. Examination showed akinesia and rigidity. MMSE was 9/30. Routine biological investigations were unrevealing, with no changes on brain CT. EEG showed slow left activities. Four months later, the patient was admitted to the hospital bedridden in the course of continuous and progressive decline. He was alert but unresponsive to voice. Examination disclosed rest myoclonus increased by noise, with no pyramidal features or focal signs. There was biological evidence of dehydration, with elevated natremia and creatinemia. CSF was germ-free, without pleocytosis. EEG showed slow delta activity without periodic discharges. The patient died 5 days after admission. Autopsy disclosed numerous senile plaques, neurofibrillary tangles, and cortical and subcortical Lewy bodies without spongiform changes.

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Comment

Both of these case reports are of particular interest because the patients evolved very rapidly to a fatal outcome (8 and 22 months) and had myoclonus in the late stage. Mean duration of DLB ranges from 5 to 8 years,2 although a 22-month duration has been described.3 Myoclonus is uncommon in DLB and was not mentioned in the first clinical descriptions.4,5 Although not excluded, it is not a feature essential to or supportive of the diagnosis of DLB.1 Myoclonus in patients with a rapidly progressive form of dementia raises the suspicion of Creutzfeldt-Jacob disease. Once a prion disease is ruled out, DLB should be considered in such patients.

McKeith IG, Galasko D, Kosaka K 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—  1124
 
Gomez-Tortosa E, Ingraham AO, Irizarry MC, et al: Dementia with Lewy bodies. J Am Geriatr Soc 1998; 46:1449—  1458
 
Litvan I, McKee A: Clinicopathologic case report: dementia with Lewy bodies (DLB). J Neuropsychiatry Clin Neurosci  1999; 11:107—112
[PubMed]
 
Ala TA, Yang KH, Sung JH, et al: Hallucinations and signs of parkinsonism help distinguish patients with dementia and cortical Lewy bodies from patients with Alzheimer's disease at presentation: a clinicopathological study. J Neurol Neurosurg Psychiatry  1997; 62:16—21
[CrossRef] | [PubMed]
 
Kosaka K: Diffuse Lewy body disease in Japan. J Neurol  1990; 237:197—204
[CrossRef] | [PubMed]
 
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References

McKeith IG, Galasko D, Kosaka K 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—  1124
 
Gomez-Tortosa E, Ingraham AO, Irizarry MC, et al: Dementia with Lewy bodies. J Am Geriatr Soc 1998; 46:1449—  1458
 
Litvan I, McKee A: Clinicopathologic case report: dementia with Lewy bodies (DLB). J Neuropsychiatry Clin Neurosci  1999; 11:107—112
[PubMed]
 
Ala TA, Yang KH, Sung JH, et al: Hallucinations and signs of parkinsonism help distinguish patients with dementia and cortical Lewy bodies from patients with Alzheimer's disease at presentation: a clinicopathological study. J Neurol Neurosurg Psychiatry  1997; 62:16—21
[CrossRef] | [PubMed]
 
Kosaka K: Diffuse Lewy body disease in Japan. J Neurol  1990; 237:197—204
[CrossRef] | [PubMed]
 
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