REM behavior disorder is a common sleep abnormalities that may prodrome the cognitive and motor manifestations of DLB

4. ◘ MAE is a 67-years old male patient, works as a peasant.
◘ The patients had a long history of chronic constipation, frequent exposure to
pesticides, he is HCV positive and received a course of interferon.
◘ About 4 years ago, the patient suddenly got up sleep just before sunrise,
shouting and screaming; (save me, the train is running behind me, it will hit
me). The patient got out the bed, open the door of the room then the house,
jump downstairs, and fall on the ground with resultant facial and chest wall
bruises and fractured right clavicle.
◘ The patient awakened after minutes and recalled that he had a dream with
the same story.

5. ◘ Similar dream encasement behaviors recurred every now and then, but with
different scenarios including running from wild animals or chasing by
strange people. The common factors in these nocturnal events were that
they were scary, frightening and occur several hours after sleep onset.
◘ The family doctor diagnosed the attacks as hepatic encephalopathies based
on a history of HCV positive in lab investigations and mild hepatic
periportal fibrosis in U/S.
◘ Later, the patient sought neurological consultation, diagnosed as nocturnal
epilepsy and received 1200 mg/day oxcarbazepine with persistence of the
nocturnal events every several weeks.

6. ◘ 2-year ago, the neurologist recommended PSG which showed delayed REM
latency, increased WASO, reduced REM % of TST and increased REM
sleep without atonia (RSWA) index.
◘ At this time, the patient was diagnosed idiopathic RBD and received 1 mg
clonazepam, 50 mg trazadone and 25 mg agomelatine with significant
reduction in the severity and frequency of the dream encasement
behaviors.

7. ◘ 1.5-years ago, the patient started to develop cognitive dysfunctions
interfering with the performance of his daily activity with prominent
visual hallucinations of seeing strange persons who want to harm hem,
speech difficulties, infidelity delusions, getting lost in familiar places with
difficulty in returning home and misplacing of things in the house.
◘ 1-year ago, the patient developed extrapyramidal manifestations in the form
of rigidity and bradykinesia.
◘ The cognitive and extrapyramidal manifestations showed some waxing and
waning of severities.

9. 510 minTotal sleep Time
14.6 minSleep latency
130 minWake after sleep onset
74%Sleep efficiency
135 minREM latency
8.2 %REM % TST
48.5RSWA index
10.4 %N1 % TST
45.6 %N2 % TST
35.8 %N3 % TST
12 / hourApnea – hypopnea index
11.2 /hourArousal index
13.9 / hourSleep stage transition index
14.3 / hourPLMs index
17.1 / hourSnore index
N
N
N
N
N

10. ◘ Hypnogram of the studied case showing repeated arousals, mild delayed
REM sleep latency, reduced REM % of TST and increased N3 % of TST.

15. 120
The number of epochs had > 15 sec tonic or phasic RSWA
The total REM sleep epochs
X
120 = No. of Epochs / hour (Epoch = 30 sec.)
◘ DelRosso LM, Chesson Jr. AL, Hoque R. Characterization of REM Sleep without Atonia in Patients with
Narcolepsy and Idiopathic Hypersomnia using AASM Scoring Manual Criteria. Journal of Clinical
Sleep Medicine. 2013; 9(7):675–80. DOI: http://dx.doi.org/10.5664/ jcsm.2836 .

18. ◘ REM sleep is known as paradoxical
or desynchronized sleep which is
characterized by intense neuronal
activities, rapid saccadic eye
movements (< 500 msec) and
skeletal muscles atonia.

19. ◘ More than 80% of dreams occur
during REM sleep which are
usually emotionally charged,
unrealistic, well-formed visual
hallucinations, and could be
recalled.

20. ◘ RBD is a parasomnia characterized
by loss of normal REM atonia and
encasement of vivid frightening
dreams resulting in injury to the
patient and/or bed-partner.

21. (1) Mild RBD:
- RBD ≤ 1/month and causes mild discomfort
for the patient and bed-partner.
(2) Moderate RBD:
- >1/month but <1/week and is associated with
physical discomfort to the patient or bed-
partner.
(3) Severe RBD:
- RBD >1/week and is associated with physical
injury to the patient or bed-partner.

23. ◘ RBD is the most common
parasomnias in DLB & PDD which
usually precedes the motor
manifestations.
◘ RBD is caused by extranigral
deposition of α-synuclein and
Lewy Bodies in brainstem nuclei
responsible for REM sleep
regulation (pontine REM-on and
REM-off nuclei).

26. ◘ Essential for a Diagnosis of DLB:
- Progressive cognitive decline sufficient to interfere with the usual daily activities.
- Early deficits on tests of attention, executive function, and visuo-perceptual ability.
- Memory impairment may not necessarily occur in the early stages but is usually
evident with progression.
◘ Core Clinical Features: (The first 3 typically occur early and may persist throughout the course)
(1) Fluctuating cognition with pronounced variations in attention and alertness.
(2) Recurrent visual hallucinations that are typically well formed and detailed.
(3) RBD, WHICH MAY PRECEDE COGNITIVE DECLINE.
(4) One or more spontaneous cardinal features of parkinsonism (bradykinesia, rest
tremor, or rigidity).

27. ◘ Supportive Clinical Features:
- Severe sensitivity to antipsychotic agents; postural instability; repeated falls; syncope or
other transient episodes of unresponsiveness; severe autonomic dysfunction, e.g.,
constipation, orthostatic hypotension, urinary incontinence; hypersomnia;
hyposmia; hallucinations in other modalities; systematized delusions; apathy,
anxiety, and depression.
◘ Indicative Biomarkers:
(1) Reduced dopamine transporter uptake in basal ganglia demonstrated by SPECT or
PET.
(2) Abnormal (low uptake) 123iodine-MIBG (meta-iodo-benzyl guanidine) myocardial
scintigraphy.
(3) PSG CONFIRMATION OF REM SLEEP WITHOUT ATONIA.

28. ◘ Supportive biomarkers:
- Relative preservation of medial temporal lobe structures on CT/MRI scan.
- Generalized low uptake on SPECT/PET perfusion/metabolism scan with reduced occipital
activity ± the cingulate island sign on FDG-PET imaging.
- Prominent posterior slow-wave activity on EEG with periodic fluctuations in the pre-
alpha/theta range.
◘ DLB Red Flags:
(a) Presence of any other physical illness or brain disorder including cerebrovascular disease,
sufficient to account in part or in total for the clinical picture, although these do not
exclude a DLB diagnosis and may serve to indicate mixed or multiple pathologies
contributing to the clinical presentation, or
(b) If parkinsonian features are the only core clinical feature and appear for the first time at a
stage of severe dementia.

29. ◘ Probable DLB:
(a) Two or more core clinical features of DLB are present, with or without the presence of
indicative biomarkers, or.
(b) Only one core clinical feature is present, but with one or more indicative biomarkers.
Probable DLB should not be diagnosed on the basis of biomarkers alone.
◘ Possible DLB:
(a) Only one core clinical feature of DLB is present, with no indicative biomarker evidence, or
(b) One or more indicative biomarkers is present but there are no core clinical features.
◘ DLB should be diagnosed when dementia occurs before or concurrently with
parkinsonism. The term PDD should be used to describe dementia that occurs in
the context of well-established Parkinson disease.

31. ◘ RBD is a common early manifestation of
DLB which greatly disturb the patient's
and caregiver quality of life and need
special attention and orientation to
avoid misdiagnosis, faulty management,
and unnecessary medications if it
prodromes the cognitive
manifestations.

32. ◘ When the RBD prodrome the cognitive
impairment, you are most likely
confronted with a case of
α-synucleinopathies (Dementia with
Lewy bodies or PD dementia) rather
than tauopathies (AD).

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