DIFFUSE LEWY BODY DISEASE

- Also known as dementia with Lewy bodies (DLB), Lewy body dementia or diffuse Lewy body disease
- Lewy bodies are clump of alpha-synuclein and ubiquitin proteins in neurons
- Loss of cholinergic neurons results in cognitive dysfunction and loss of dopamine neurons results in
parkinsonism

Management
- Reassure someone & support them and orient them to reality
- Exercise during the day and reduce daytime napping
- Medications can be beneficial
- Consult health professionals (neurologist, occupational therapists and physiotherapist)

Manifestations
➢ Parkinson’s disease with dementia = If dementia occurs > 1 yr after other symptoms of Parkinson’s disease
➢ Diffuse Lewy Body disease = if dementia occurs within the 1st year of parkinsonism
➢ Tend to progress much more rapidly than Parkinson’s disease
- Fluctuating cognition and alertness
- Visual hallucinations in 70% (people or animals)
- Orthostasis and frequent falls
- Motor manifestations of parkinsonism (rigidity, tremor less common than in PD)
- Onset of dementia within 1 year of symptoms of parkinsonism
- Rapid eye movement sleep behavior disorder (RBD) is very common (50-80%)
- Life-threatening rigidity with dopamine blockers and may develop malignant neuroleptic syndrome, so pt is
very sensitive to dopamine blockers agents (duh)
 FETAL ALCOHOL SYNDROME Therapeutic managements
- Minimize prenatal exposure to teratogens
- leading cause of preventable birth defects and developmental delay in children
- Food fortification with folic acid
- Incidence rate: 2 per 1000 newborn
- Multivitamins
- Worldwide: 1.9/1000 live births
- Immunization against rubella prior to pregnancy
- Predictable patterns of fetal and neonatal dysmorphogenesis are attributed to severe, chronic alcoholism in
- Education
women who continue to drink heavily during pregnancy
Medical/surgical management
Features
➢ Fetal surgery
➢ Pre and postnatal growth restriction
a. Open fetal surgery
➢ CNS involvements
o Completely opening the uterus to operate on the fetus
- Cognitive challenge, microcephaly (small head), cerebral
b. Minimally invasive fetoscopic surgery
palsy
o Uses small incisions and is guided by fetoscope and sonography
- Facial features (short palpebral fissures and thin upper
c. Percutaneous fetal therapy
lip)
o Placing a catheter under continuous ultrasound guidance
Symptoms ➢ Termination of affected

➢ CNS features
- Microcephaly
- Mental retardation
- Hyperactivity
- Impaired language development
- Delayed development of gross motor skills like rolling over, sitting up, crawling and walking
- Delayed in development of fine motor skills such as grasping objects with the thumb and index fingers and
transfering objects from one hand to the other
- Seizures