Creutzfeldt-Jakob disease

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Manifestation of Novel Social Challenges of the
European Union
in the Teaching Material of
Medical Biotechnology Master’s Programmes
at the University of Pécs and at the University
of Debrecen
Identification number: TÁMOP-4.1.2-08/1/A-2009-0011
Manifestation of Novel Social Challenges of the
European Union
in the Teaching Material of
Medical Biotechnology Master’s Programmes
at the University of Pécs and at the University
of Debrecen
Identification number: TÁMOP-4.1.2-08/1/A-2009-0011
Márta Balaskó and Gyula Bakó
Molecular and Clinical Basics of Gerontology – Lecture
17
NEUROLOGICAL
DISORDERS IN THE
ELERLY
PART II
The most common agingassociated neurological
disorders (outline)
TÁMOP-4.1.2-08/1/A-2009-0011
• Disorders of cerebral blood flow
(stroke)
• Neurodegenerative diseases
affecting motor (and later cognitive)
functions
(e.g. Parkinson’s diasease)
• Other, more frequent neurological
disorders also present in old
individuals with high prevalence:
- myasthenia gravis
- headache
- dizziness (vertigo) in the elderly
Neurodegenerative diseases
affecting motor functions:
classification
TÁMOP-4.1.2-08/1/A-2009-0011
Earlier definitions: extrapyramidal
disease, diseases of the basal ganglia
Types:
• Hypokinetic syndromes
- Parkinson’s disease
- Symptomatic parkinson syndromes
- Multiple system atrophy
• Hyperkinetic syndromes
- Huntington chorea
- Creutzfeldt-Jakob disease
TÁMOP-4.1.2-08/1/A-2009-0011
Neurodegenerative diseases
affecting motor functions
Parkinson’s diasease
Movement-related disease of unknown
origin including shaking, rigidity,
slowness of movement and difficulty
with walking with dementia in the later
phases.
Huntington-chorea
A neurodegenerative autosomal dominant
genetic disorder that affects muscle
coordination (with abnormal involuntary
writhing movements = chorea) with
cognitive decline and dementia.
Parkinson’s disease:
etiology prevalence
TÁMOP-4.1.2-08/1/A-2009-0011
• A disease with progressive movement disorder
• Special progressive cell death of the
substantia nigra – with consequent dopamine
deficiency
• The origin of the cellular damage is unknown
Risk factors:
• AGE
• Noxious effects (CO poisoning, infections,
pesticides)
• Mutations of the tau-gene
Prevalence:
• 100-200/100,000 population
TÁMOP-4.1.2-08/1/A-2009-0011
Neurotransmitter imbalance
in Parkinson’s disease
Balance between
dopamine
and
glutamate/acetylcholine
Glutamate
Acetylchol
Dopamine
Imbalance as a result
of
dopamine deficiency
Glutamate
Acetylchol
Dopamine
TÁMOP-4.1.2-08/1/A-2009-0011
Parkinson’s disease: early
symptoms
Symptoms:
• Asymmetry: one-sided motor symptoms
- tremor (at rest and aggravated by sustained
effort),
- rigidity of muscles (cogwheel phenomenon
upon passive movement of limbs),
- hypo- and bradykinesis (difficulty in
initiating movement and getting frozen
during the course of it),
- lack of expression in the face (mask-like
face, diminished eye blinking)
- postural abnormalities
- slow and monotonous speech
• Upon levodopa administration symptoms
TÁMOP-4.1.2-08/1/A-2009-0011
Parkinson’s disease: late
symptoms
Motor functions
• postural instability
(after 8-10
years)
Autonomic functions
• seborrheic oily skin
• orthostatic hypotension
• gastrointestinal disorders (dysphagia,
constipation)
• sphincter disturbances
• impotence
• enhanced sweating
Cognitive decline
TÁMOP-4.1.2-08/1/A-2009-0011
Secondary (symptomatic)
parkinsonism
Different types of secondary Parkinsonism show
somewhat similar symptoms as Parkinson’s
disease, but they do not react to levodopa. The
progression is frequently more rapid.
• Infections: complications of
viral
encephalitis
• Atherosclerosis of cerebral vessels: vascular
Parkinsonism
• Toxins: Carbon monoxide, Manganese
• Drugs: neuroleptic drugs, reserpine,
metoclopramide, methyldopa
• Metabolic disorders: parathyroid disorders,
cerebral hypoxia
TÁMOP-4.1.2-08/1/A-2009-0011
Huntington disease (chorea)
Characteristics:
• 5-10/100,000 population
• Autosomal dominant inheritance (huntingtin,
chromosome 4)
• Onset: 40-50 years of age
• Mean survival does not exceed 15 years
• Main symptom: involuntary abnormal choreiform
writhing movements
• Psychiatric symptoms are present from the
beginning (affective disorders, schizoform
symptoms)
• No available treatment
TÁMOP-4.1.2-08/1/A-2009-0011
Creutzfeldt-Jakob disease
Characteristics:
• Prevalence: rare: 1 / 1,000,000 population
• Cause: prion (infectious agent composed of
protein in a misfolded form)
• Onset is usually about the age of 60 years
(50-70)
• Triade of symptoms:
- dementia (starts with failing memory, mental
deterioration)
- characteristic EEG findings
- myoclonus
• Other symptoms occur during progression:
involuntary movements and muscle weakness,
The most common agingassociated neurological
disorders (outline)
TÁMOP-4.1.2-08/1/A-2009-0011
• Disorders of cerebral blood flow
(stroke)
• Neurodegenerative diseases
affecting motor functions (e.g.
Parkinson’s disease)
• Other, more frequent neurological
disorders also present in old
individuals with high prevalence:
- myasthenia gravis
- headache
- dizziness (vertigo) in the elderly
TÁMOP-4.1.2-08/1/A-2009-0011
Myasthenia gravis
General characteristics
• An autoimmune neuromuscular disease
affecting acetylcholine receptors
• Leading symptoms: fluctuating muscle
weakness and fatiguability that
ameliorates upon rest
• The disease affect predominantly:
- ocular (ptosis),
- bulbar (swallowing, chewing, speech)
- facial muscles (lack of facial
expression)
- Neck
TÁMOP-4.1.2-08/1/A-2009-0011
Pathomechanism of myasthenia
gravis
A nerve
ending
Nerve
endings
spread along
muscle
Nerve
Antibody
Muscle
Surface of
muscle
fiber
Some receptors
are blocked or
damaged by
antibodies
Acetylcholin
e is
released
from nerve
Neuromuscular
ending
junction (gap
between nerve
and muscle)
Receptor on
surface of
muscle fiber
Some receptors are
stimulated by
acetylcholine
TÁMOP-4.1.2-08/1/A-2009-0011
Myasthenia gravis in the
elderly
Special characteristics in the elderly
• It progresses to a severe, fatal
disease more frequently sooner
• Complete remission occurs rarely
• Crises presents higher risk for lethal
outcome
• Immunosuppressive treatment is more
frequently needed
• More frequent drug side-effects
• Comorbidities (e.g. hypothyroidism 15%)
TÁMOP-4.1.2-08/1/A-2009-0011
Head-aches in the elderly
Primary (60-70% of all head-aches)
• Migraine (much less prevalent than in the
young)
• Tension head-ache (frequent)
• Cluster head-ache (predominantly in males,
above 60 years)
Secondary (they are more prevalent in the
elderly)
• Intracranial vascular diseases
• Intracranial tumor, inflammation
• Other disease-associated forms (e.g. earthroat and nose, diseases of the locomotor
system)
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Dizziness in the elderly
Characteristics:
• Very frequent complaint in the elderly
• 30% of people older than 65 years
experience dizziness in some form,
increasing to 50% in the very old
(older than 85 years)
• Above 80 years of age: females 66%,
males 33%
• It is often associated with
depression,
• Anxiety increases the prevalence
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Dizziness in the elderly:
causes
• Vertebrobasilar circulatory disorders
(the most frequent cause among the
elderly)
• Degenerative diseases or ischemia
affecting the vestibular organ
• Degeneration of mechanoreceptors in
the neck region
• Diminished physical activity due to
some other disease, immobilization
• Oscillations of blood pressure
• Exsiccosis (hypovolemia)
The most common agingassociated neurological
disorders (outline)
TÁMOP-4.1.2-08/1/A-2009-0011
• Disorders of cerebral blood flow
(stroke)
• Neurodegenerative diseases affecting
motor (and later cognitive) functions
(e.g. Parkinson’s disease)
• Other, more frequent neurological
disorders also present in old
individuals with high prevalence:
- myasthenia gravis
- headache
- dizziness (vertigo) in the elderly
TÁMOP-4.1.2-08/1/A-2009-0011
Peripheral neuropathy in
the elderly
Peripheral neuropathy is a widespread damage of
the peripheral nervous system.
Prevalence: 2400/100,000 (2.4%), rising with
age to 8000/100,000 (8%). In the elderly,
losses of vibratory sensation in the lower
extremities and ankle reflexes are common.
Causes: (40-70% idiopathic or autoimmune):
• diabetes mellitus (most frequent known cause
in Europe)
• aging, alcoholism, HIV, toxin exposure,
metabolic abnormalities, vitamin B12, B1
deficiencies, side effects of drugs
Consequences: sensory, motor, autonomic
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