DLB: 3 cases

Case 1. Female Age 75

Initial presentation 2012:
Some minor forgetfulness which ended in a relative acute
confusion related to husbands acute accidental death. Already
before there had in retrospect been some accounts of mild
visual hallucinations (VH).
Markedly Reduced visus

Symptoms
The main symptoms were VH, consisting of seeing
small children and people standing and listening, but
not saying anything. These tended to worsen, and
became more disturbing and occasionally frightening.
Later she also developed paranoid delusions, and
finally lead to acute psychiatric hospitalization with
police (!). Treatment with haloperidol and later
risperidone was initiated, which lead to severe side

Other illnesses
Osteoarthritis, leg pain, depression, hearing and visual impairment
all contributed to the overall condition and made DLB diagnosis
more difficult.
Biomarkers

ECG, CSF analysis, CT (vascular changes

including in basal ganglia).MRI Blood tests.
The Dat scan showed pathological uptake
Drug treatment:
Antihypertensives, ASA; DLB symptoms later were
antipsychotics, clearly a suboptimal potentially dangerous
alternative due to misdiagnosis.
The Dat scan results ascertained the diagnosis , and lead
to changing from antipsychotic to antidementia drug
treatment. First rivastigmine, and subsequently
memantine, which showed dramatic cognitive

DaTscan: [123I]FP-CIT SPECT

AD

13-03-15

DLB

Department of Clinical Medicine

PD

Key learning points

This case shows the dramatic consequences of misdiagnosing
the psychotic symptoms in DLB as a psychotic disorder rather
than DLB, which lead to high-dose antipsychotics, even
haloperidol, and delayed treatment with antidementia drugs.
Late-onset VH should always lead to suspicion of DLB. The
antipsychotic treatment lead to parkinsonism which was then
difficult to interpret in relation to DLB.
First psychiatrists did not diagnose DLB, neither did geriatricians,
who suspected cerebrovascular disease and confusion but not
DLB.

Case 2 HH (1999) 82 yr old female,

previous teacher.
Previously; healthy, intelligent, artistic, humble person.
Presented with change of personality (apathy, crude behaviour, egocentric and
sarcastic), memory and planning deficits, neglect of selfcare, depressive symptoms.
Normal language.
Prior to assessment treated with sertraline with some effect.
Cognitive screening: MMSE 27; Mattis Dementia Rating scale (DRS) 137/144
NPE: Fluency: phonetic fluency very poor, design fluency good. Deficits of verbal
memory (recall, retention relatively preserved), poor executive functioning and
psychomotor speed)
Psychiatric: NPI- Aggression 8, depression 12, Irritability 8
Neurology: mild asymmetric tremor, indifferent plantar reflex right side. Normal blood
tests.

Year 2000
Neurologist diagnosed parkinsonism (tremor, gait),
trial with levo-dopa; no clear response.
MMSE 23. Visuospatial impairment.
CT: Some central and cortical atrophy, mainly temporal;
some white-matter lesions
Trial with rivastigmine; some response
MMSE 23-24-26-23

Further course
2001: Admitted psychiatric hospital (lying on floor, screaming,
wanting to kill herself)
Perfusion SPECT: temporal and parietal hypoperfusion; normal
frontal/Occipital perfusion.
Worsening gait. Admitted nursing home.
2002. Wheelchair. MMSE 16. Delusional (nurses will kill me),
hallucinations.
2003: Worsening overall, MMSE 20. Motor worsening, unclear
diagnosis.
2004: Died

Died 2004-autopsy
No Lewy bodies.
No AD changes
Frontal lobe: Fairly
normal

Learning:
Unusual case
Diagnosis not clear; not even after autopsy
FTD with parkinsonism, can have abnormal Dat scan

Case 3 TG-70yr old man with

parkinsonism
Premorbid: over-average intelligence, 19 yrs schooling
Family history: Mother AD late; father died age 72- 20 years with balance
and language problems; no diagnosis- but multiple scleroris had been
discussed. 8 siblings. Sister with AD; brother with PD. (Another brother
seen by me with very subtle cognitive and motor changes, DAT scan
interpreted normal).
Genetic analyses of most AD and PD genes performed without evidence of
known mutations.
Atrial fibrillation; warfarin treatment.
On vacation in Spain 2008, olecranon-abscess, developed severe
delirium.

First seen 2008

2-3 yrs parkinsonistic symptoms
Sleep disturbances (interrupted sleep, not insomnia,
sleep wandering).
Subtle behavioural changes: apathy, dyscalculia,
reduced instrumental daily functioning.
Visual hallucinations, no auditory

Exam
Exam: left-sided dominant parkinsonism, bradykinesia, postural
change; no tremor
MRI: Age-associated changes.
CSF: Normal
DAT scan: asymmetric reduced uptake of putamen and also
caudate, most pronounced right side.
EEG: drowsy, no pathology
Drugs: Started with donepezil (good response); clomethiazol for
sleep with improvement.

Course:
Annual MMSE: 28-29-30-30;
Annual CDR-SB: 2-4.5-6-4
2008: TMA 96, TMB 164; CVLT 1-5: 29; NPI-2: 12
Year 1: UPDRSIII: 14; TMTA 102; TMTB 219; CVLT1-5: 49
Year 2: UPDRS 25;
Died 2015-awaiting pathology

Learning
Typical presentation
But unusually stable course, in particular on global
screening (MMSE)
Showed progressive parkinsonism and executive decline