Elsevier

The Lancet Neurology

Volume 4, Issue 10, October 2005, Pages 605-610
The Lancet Neurology

Fast track — Articles
Visual hallucinations in the diagnosis of idiopathic Parkinson's disease: a retrospective autopsy study

https://doi.org/10.1016/S1474-4422(05)70146-0Get rights and content

Summary

Background

For many years, visual hallucinations (VH) in idiopathic Parkinson's disease (PD) were thought to be a complication of antiparkinsonian treatment. The cause of VH is now thought to be nerve-cell loss and Lewy-body pathology in the ventral-temporal regions of the brain. However, the use of VH as a clinical sign of PD has not been investigated.

Methods

788 cases with parkinsonism archived at the Queen Square Brain Bank for Neurological Diseases were identified for study. Patients had not been given standardised antemortem assessments. Clinical records were assessed for reports of VH. The incidence of VH in pathologically diagnosed cases was calculated, and factors affecting onset of VH were investigated.

Findings

VH occurred in 50% (221/445) of patients with PD, in 73% (32/44) with dementia with Lewy bodies (DLB), and in only 7% (18/255) of patients with non-Lewy-body parkinsonism. The specificity of VH for Lewy-body parkinsonism (PD and DLB combined) was 92·9% (95% CI 89·1–95·8) and the positive predictive value was 93·4% (89·7–95·8). VH were associated with cognitive dysfunction (hazard ratio 5·62, 3·37–9·35), autonomic dysfunction (3·13, 1·77–5·52), axial rigidity (2·22, 1·26–3·85) within the first 2 years of disease onset and also age of onset (1·05, 1·03–1·07). In PD, the onset of VH typically occurred in the second half of the disease course, and time to onset of VH was only weakly correlated with use of selegiline (Spearman's rho 0·22, p=0·005) and ergot dopamine agonists (0·24, p=0·006) but not correlated with use of levodopa, amantadine, or anticholinergic drugs.

Interpretation

The presence of VH is helpful in the differentiation of PD from other non-Lewy-body causes of parkinsonism. We propose that VH be added, as a supportive criterion, to the operational clinical criteria for the diagnosis of PD.

Introduction

Visual hallucinations (VH) and disturbances of visual perception occur in several neurodegenerative diseases, including Parkinson's disease (PD) and dementia with Lewy bodies (DLB). In patients with PD the prevalence of VH has been reported to be between 6% and 60%.1, 2 VH in patients with Lewy body pathology are thought to be secondary to pathology in the basolateral nucleus of the amygdala and parahippocampus, and related to α synuclein pathology in the frontal cortex.3, 4, 5 The time from disease onset to first VH has been related to density of Lewy bodies in the parahippocampal and inferior temporal cortices.5 In patients with neurodegenerative dementias, the presence of VH is helpful in differentiating DLB from frontotemporal dementias and progressive supranuclear palsy, but not from Alzheimer's disease.6 Although the prevalence of VH has not been systematically studied in other pathologically diagnosed bradykinetic-rigid syndromes, there are some clinical reports of VH in multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration.7, 8, 9, 10 In these disorders, Lewy bodies do not typically occur in the frontal or temporal cortices, which suggests that when VH are present they are due to different mechanisms than in Lewy body parkinsonism (LBP). This distinction might be clinically helpful when diagnosing patients with bradykinetic-rigid syndromes, particularly for differentiating between PD and non-LBP. We aimed to investigate the relation between pathological diagnosis of a bradykinetic-rigid syndrome and VH and the relation between VH and other clinical factors, such as drugs.

Section snippets

Patients

We studied cases with bradykinetic-rigid syndrome or parkinsonism archived at the Queen Square Brain Bank for Neurological Diseases. Donors were from the UK and died between 1988 and 2003. Cases had been diagnosed before death as well as pathologically confirmed; all met widely accepted pathological criteria for diagnosis.11, 12, 13, 14, 15, 16 Of 788 cases identified, 473 had PD, 44 had DLB (where cognitive dysfunction was recorded within the first year of disease onset), 127 had progressive

Results

44 cases were excluded from the study because of insufficient clinical data: 28 with PD, seven with progressive supranuclear palsy, five with multiple system atrophy, two with Alzheimer's disease, and two with vascular parkinsonism. Of the 744 cases included (table 1), 272 (37%) had VH. In four patients with progressive supranuclear palsy and three patients with multiple system atrophy VH were related only to dopaminergic drugs and ceased on withdrawal of the offending drug. These patients were

Discussion

VH are a useful clinical diagnostic feature in patients with PD. VH are a common symptom in patients with LBP and rarely occur in patients with other bradykinetic-rigid syndromes. The presence of VH predicted LBP with 93% accuracy. VH occurred in 50% of patients with PD, and in almost all of these VH occurred in the second half of the disease course. VH were only weakly correlated with use of selegiline and ergot dopamine agonists; they were not correlated with use of levodopa, amantadine, or

References (31)

  • OL Lopez et al.

    Accuracy of four clinical diagnostic criteria for the diagnosis of neurodegenerative dementias

    Neurology

    (1999)
  • K Nagaoka et al.

    A case of corticobasal degeneration presenting with visual hallucination

    Rinsho Shinkeigaku

    (2004)
  • Y Shimo et al.

    A-56-year-old woman with parkinsonism, whose mother had Parkinson's disease

    No To Shinkei

    (2001)
  • AJ Lees et al.

    The use of lisuride in the treatment of multiple system atrophy with autonomic failure (Shy-Drager syndrome)

    J Neurol Neurosurg Psychiatry

    (1981)
  • D Aarsland et al.

    Neuropsychiatric symptoms of patients with progressive supranuclear palsy and Parkinson's disease

    J Neuropsychiatry Clin Neurosci

    (2001)
  • Cited by (0)

    View full text