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Lewy Body Dementia : Symptoms, Diagnosis, and Treatment

Apr 12, 2024

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Understanding The Clinical Features Of Lewy Body Dementia

Multi System Atrophy

Exploring The Neuropathology Of Lewy Body Dementia

Intracytoplasmic and Intraneuronal inclusions 

Diagnosis And Workup

Treatment Strategies For Lewy Body Dementia

Conclusion

Symptoms, Diagnosis, and Treatment of Lewy Body Dementia

Among the neurodegenerative diseases, Lewy body dementia (LBD) presents a significant difficulty because of its wide range of symptoms, many of which are also present in other conditions, such as Alzheimer's and Parkinson's diseases. Taking its name from the brain's alpha-synuclein aggregation, LBD has distinct difficulties in terms of diagnosis and treatment. We examine the clinical characteristics, underlying neuropathology, diagnosis techniques, and possible treatment options of Lewy body dementia in this extensive guide.

Understanding The Clinical Features Of Lewy Body Dementia

Dementia with Lewy bodies and Parkinson's disease with dementia are two extremes of the spectrum on which LBD presents. Visual hallucinations are one of the main characteristics of LBD, and they can be upsetting for both patients and caretakers. Patients may also have REM behavioral disorder (RBD), which is characterized by dream theater when they are asleep. Parkinsonism, cognitive fluctuations, delusions, orthostatic hypotension, and spells of bewilderment are other typical symptoms.

Notably, symptoms such as loss of smell and constipation may occur prior to the onset of Parkinson's disease, highlighting the complexity of LBD's clinical presentation.

Also Read: Chronic Fatigue Syndrome (CFS) and Myalgic Encephalomyelitis (ME): Diagnosis, and Treatment

Multi System Atrophy

Features 

Mild cognitive impairment LB pathology 

Mild cognitive impairment Alzheimer’s disease pathology

Domain Affected

  • Executive functions (organizing, planning and thinking)
  • Attentional and visuospatial deficit
  • Impaired memory
  • semantic naming

Fluctuations in cognition

Frequent

Rare

Sleep issues

REM behavioral disorder Frequent

Frequently awakening

Insomnia

Visual hallucinations

Frequent

Rare

Polysomnogram

RBD: EMG with respect to the peripheral extremities will show the physical movement 

No specific activity

CSF

Alpha synuclein levels are very less

  • Beta amyloid values are reduced.
  • Phosphorylated tau will be increased

MRI

Atrophy of the amygdala 

Parahypocampus will be affected 

18 FDG PET

Decreased metabolism in the occipital cortex

Decreased activity of the parietal and temporal lobe 

Amyloid PET

-

+

MiBG myocardial scintigraphy

Post ganglionic sympathetic denervation 

Not seen

PET DOPA

Decreased activity of the basal ganglia (Dopamine)

Normal 

CSF

Decrease in the alpha synuclein

Decrease in Abeta amyloid

Also Read: Image Based Questions On Neurology


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Exploring The Neuropathology Of Lewy Body Dementia

The cause of neurodegenerative dementia is dysbiosis Alpha syn aggregation and oxidative stress propagate from the lower to the cerebral regions. The heart may also be involved. When the inclusions enter the brain, they are intracytoplasmic. Alpha synuclein is the protein that makes up Lewy bodies.

Intracytoplasmic and Intraneuronal inclusions 

Among the stains is PAS reagent,ubiquitin. The enteric nervous system is the cause of Parkinson's disease. Scent bulbs travels through the vagus nerve to reach the heart from the anterior nervous system.
The sympathetic pathway's post-ganglionic fibers will become denervated. Examination - Myocardial Scintigraphy using MiBG.
There is involvement in the lower brainstem region. Hypotension in orthostatic posture Putamen and Substansia nigra are implicated. 

The amygdala was later affected by the disease (Limbic system). The cognitive impairment is caused by cerebral cortex involvement.

Also Read: Prion Disease- Pathogenesis, Diagnosis and Management

Diagnosis And Workup

MRI brain distinction- Atrophy of Multiple Systems, Amygdala atrophy.
The polysomnographic method- Less tone in the muscles and little movement during the REM period in a normal person while electrodes for LBD will detect electrical activity resulting from the tearing of limbs and legs. Alpha synuclein levels dropped in the CSF research.
Test: Conversion caused by trembling in real time. Myocardial scintigraphy with MiBG shows that the spread of prion causes post-ganglionic sympathetic denervation. PET scan- One observes hypometabolism., more costly.

Also Read: Neuromyelitis Optica: Revealing the Mystery 

Treatment Strategies For Lewy Body Dementia

Treatment

Description

Cholinergic deficit

  • Rivastigmine or Donepezil
  • It will reduce the attention deficit.

Clozapine

  • Risk of agranulocytosis

Pimavanserin

  • Atypical antipsychotic drug
  • Inverse agonist of serotonin receptor (Effect opposite to agonist)
  • Decrease the hallucinosis component

RBD

  • Melatonin is recommended (Supportive care)

Escitalopram

  • To treat mood and anxiety

Orthostatic hypertension

  • Change in the BP in supine and standing position. 
  • Tilt table test is performed.
  • Midodrine (Alpha 1 agonist): Causes vasoconstriction.
  • 30-degree elevation of head of the bed
  • Fludrocortisone
  • Increase the amount of urine and salt

Physical therapy

  • To prevent Bradykinesia, rigidity
  • To prevent sudden death 

Conclusion

Lewy body dementia presents serious difficulties for individuals with the condition, their carers, and medical professionals. Through an enhanced comprehension of its neuropathology, clinical characteristics, and available treatments, we will be able to provide better assistance to those impacted by this intricate illness. The medical community's ongoing research and cooperation provide promise for better treatment strategies and diagnostic methods, which will eventually improve the quality of life for those with Lewy body dementia.

Hope you found this blog helpful for your NEET SS Medicine Neurology Preparation. For more informative and interesting posts like these, keep reading PrepLadder’s blogs.

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