CNS Pg No 575 -584
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What is the most common clinical feature of Frontotemporal Dementia?

  • Memory loss
  • Behavioral variant (correct)
  • Language difficulties
  • Visual disturbances

    • Diffuse Lewy Body Dementia is primarily associated with the MAPT gene.

    False

    At what age does Frontotemporal Dementia typically onset?

    50 - 60 years

    The most common type of Frontotemporal Dementia is the __________ variant.

    <p>behavioral</p> Signup and view all the answers

    Match the following dementias with their associated pathology:

    <p>Frontotemporal Dementia = MAPT gene on chromosome 17 Diffuse Lewy Body Dementia = Alpha-synuclein Alzheimer's Disease = Hyperphosphorylated Tau Progressive Supranuclear Palsy = Tauopathy</p> Signup and view all the answers

    What is the most important risk factor for sporadic Alzheimer's disease in the elderly?

    <p>Apo e4</p> Signup and view all the answers

    Low IQ is considered a risk factor for Alzheimer's disease.

    <p>False</p> Signup and view all the answers

    What occurs in Stage IV of dementia?

    <p>Behavioral abnormalities and personality changes</p> Signup and view all the answers

    A common defect in familial Alzheimer's disease is linked to Presenilin - 1 on chromosome ______.

    <p>14</p> Signup and view all the answers

    Match the following stages of dementia with their characteristics:

    <p>Stage I = Episodic memory loss Stage II = Naming and comprehension abnormalities Stage III = Visuo-spatial disorientation Stage IV = Behavioral abnormalities and personality changes</p> Signup and view all the answers

    Which nucleus is the only excitatory nucleus in the basal ganglia?

    <p>Subthalamus</p> Signup and view all the answers

    All output from the basal ganglia leaves through the GPe.

    <p>False</p> Signup and view all the answers

    What condition results from the degeneration of the nigrostriatal pathway?

    <p>Parkinson's disease</p> Signup and view all the answers

    The indirect pathway in the basal ganglia is indicated by ______ arrows.

    <p>red</p> Signup and view all the answers

    Match the following features with their respective pathways in the basal ganglia:

    <p>Direct Pathway = Excitatory activation of cortex Indirect Pathway = Inhibitory influence on cortex Nigrostriatal Pathway = Influences movement through D1 and D2 receptors Subthalamic Nucleus = Only excitatory nucleus in BG</p> Signup and view all the answers

    Which of the following is a common inherited condition associated with vascular dementia?

    <p>CADASIL</p> Signup and view all the answers

    Normal Pressure Hydrocephalus is characterized by atrophy of the brain.

    <p>False</p> Signup and view all the answers

    What are the typical clinical features of Normal Pressure Hydrocephalus?

    <p>Gait apraxia, urinary incontinence, and subcortical type dementia</p> Signup and view all the answers

    CADASIL is caused by a mutation in the _______ gene.

    <p>NOTCH3</p> Signup and view all the answers

    Match the features of Normal Pressure Hydrocephalus (NPH) with their descriptions:

    <p>Gait apraxia = Magnetic foot phenomenon Urinary incontinence = Inability to control urination Subcortical dementia = Type of dementia related to subcortical structures Ventriculomegaly = Enlargement of brain ventricles</p> Signup and view all the answers

    Which of the following nuclei are part of the Basal Ganglia?

    <p>Putamen</p> Signup and view all the answers

    The Subthalamic nucleus is not included in the Basal Ganglia.

    <p>False</p> Signup and view all the answers

    Name two types of movements associated with lesions in the Basal Ganglia.

    <p>Chorea and Dystonia</p> Signup and view all the answers

    The Basal Ganglia are primarily responsible for ___ and scaling of movement.

    <p>initiation</p> Signup and view all the answers

    Match the components of the Basal Ganglia with their functions:

    <p>Caudate nucleus = Cognitive part of motor activity Putamen = Initiation of movement Globus Pallidus = Regulation of muscle tone Substantia nigra = Dopamine production</p> Signup and view all the answers

    Which of the following is a characteristic of Creutzfeldt-Jakob disease (CJD)?

    <p>Spongiform changes in the cerebral cortex</p> Signup and view all the answers

    Vascular dementia is primarily caused by prion diseases.

    <p>False</p> Signup and view all the answers

    What is the most common site of infarct in large vessel disease associated with vascular dementia?

    <p>B/1 PCA lesion</p> Signup and view all the answers

    _______ is the protein associated with Alzheimer's dementia.

    <p>Αβ</p> Signup and view all the answers

    Match the type of dementia with its associated protein:

    <p>Alzheimer's = Αβ Frontotemporal dementia (FTD) = Tau Dementia with Lewy bodies (DLB) = α-synuclein Creutzfeldt-Jakob disease (CJD) = PrP<sub>sc</sub></p> Signup and view all the answers

    What is the primary component found in Lewy bodies?

    <p>α-synuclein</p> Signup and view all the answers

    Cognitive impairment in Dementia with Lewy Bodies typically begins after the onset of motor symptoms.

    <p>False</p> Signup and view all the answers

    What effect do antipsychotic medications have on symptoms of Dementia with Lewy Bodies?

    <p>Worsens symptoms</p> Signup and view all the answers

    Patients with Parkinson's disease often exhibit __________ as a common symptom.

    <p>tremors</p> Signup and view all the answers

    Match the symptoms with their corresponding disease based on the given features:

    <p>Cognitive impairment precedes motor symptoms = Dementia with Lewy Bodies Common visual hallucinations = Dementia with Lewy Bodies Improvement with L-dopa treatment = Parkinson's Disease Commonly presents with tremors = Parkinson's Disease</p> Signup and view all the answers

    What is the most important risk factor for Binswanger Disease?

    <p>Hypertension</p> Signup and view all the answers

    Cerebral Amyloid Angiopathy is primarily characterized by Aβ40 protein deposition.

    <p>True</p> Signup and view all the answers

    What type of infarct is characteristic of Binswanger Disease?

    <p>Lacunar infarct</p> Signup and view all the answers

    In patients with small vessel disease, __________ is commonly observed as a manifestation related to the corticobulbar tract.

    <p>pseudobulbar palsy</p> Signup and view all the answers

    Match the following clinical features with the affected fibres in small vessel disease:

    <p>Gait disturbances = Corticobulbar tract Urinary abnormalities = Fibres from paracentral lobule Personality changes = Corticostriate fibres Magnetic foot phenomenon = Corticospinal tract</p> Signup and view all the answers

    Which of the following treatments is considered the first line of defense in Alzheimer's disease?

    <p>Donepezil</p> Signup and view all the answers

    Aducanumab is an NMDA antagonist used in the treatment of Alzheimer's disease.

    <p>False</p> Signup and view all the answers

    What type of imaging technique is used to detect hypometabolism in Alzheimer's disease?

    <p>PET scan</p> Signup and view all the answers

    The analysis of cerebrospinal fluid (CSF) in Alzheimer's disease can show high levels of _______ and hyperphosphorylated tau.

    <p>Aβ42</p> Signup and view all the answers

    Match the following treatment options for Alzheimer's disease with their categories:

    <p>Donepezil = Cholinesterase inhibitor Rivastigmine = Cholinesterase inhibitor Memantine = NMDA antagonist Aducanumab = Monoclonal antibody</p> Signup and view all the answers

    Which of the following dementias is classified as cortical dementia?

    <p>Frontotemporal dementia (FTD)</p> Signup and view all the answers

    Mood symptoms are generally more severe in cortical dementia compared to subcortical dementia.

    <p>False</p> Signup and view all the answers

    Name one example of a subcortical dementia.

    <p>Dementia with Lewy bodies (DLB), Progressive supranuclear palsy (PSP), Normal pressure hydrocephalus (NPH), or Binswanger's disease.</p> Signup and view all the answers

    Alzheimer's disease mainly affects the __________ region of the brain.

    <p>cortex</p> Signup and view all the answers

    Match the symptoms with their respective types of dementia:

    <p>Memory Impairment = Cortical Dementia Coordination Impairment = Subcortical Dementia Agnosia = Subcortical Dementia Common Movements = Cortical Dementia</p> Signup and view all the answers

    Which of the following symptoms is normal in subcortical dementia?

    <p>Normal cognitive &amp; motor speed</p> Signup and view all the answers

    Prion diseases are characterized by the presence of nucleic acids.

    <p>False</p> Signup and view all the answers

    What is the primary pathological feature in prion diseases?

    <p>ß pleated PrPsc protein leading to spongiform degeneration.</p> Signup and view all the answers

    Study Notes

    Frontotemporal Dementia (Pick's Disease)

    • Prevalence: 70% sporadic.
    • Age of onset: Young onset dementia (50 - 60 years).
    • Progression: Rapidly progressive dementia.
    • Pathology involves the MAPT gene on chromosome 17.
    • Proteins involved: Tau (Hyperphosphorylated), Fus
    • Bilateral prefrontal cortex atrophy.
    • Most common type: Behavioral variant.
    • Symptoms: Loss of social embarrassment, dysfunction of social and emotional systems, apathy, overeating (Temporal lobe), impaired planning and judgment.
    • Memory is relatively preserved.
    • Hyperorality: Temporal lobe involvement.
    • Taupathy: Hyperphosphorylated Tau in Alzheimer's → Pick's
    • PFC (Prefrontal Cortex): Id, Superego, Ego

    Diffuse Lewy Body Dementia

    • Prevalence: 2nd most common cause of dementia.
    • Association: Part of Parkinson-plus / Atypical Parkinson's syndrome.
    • Components of Parkinson plus syndrome: Taupathies (Progressive supranuclear palsy, Corticobasal degeneration), a synuclein-opathies (Multiple system atrophy, Diffuse Lewy Body dementia)
    • Taupathy is a process involving problems with a protein called tau.
    • Diffuse Lewy body dementia is related to a synucleinopathy, specifically to Alpha-synuclein.

    Genetic Basis

    • Presenilin - 1 (chr 14): Most common defect in familial Alzheimer's disease (AD)

    • Presenilin - 2 (chr 1)

    • Apo e4 (chr 19): In late-onset dementia, ineffective degradation of AB amyloid, most important risk factor (Biomarker) for sporadic AD in elderly.

    • In early onset dementia, characteristic features include:

      • Female predominance (F > m)
      • History of head trauma/concussions
      • History of stroke
      • Metabolic syndrome
      • Hypertension
      • Diabetes

    • Low IQ, Smoking, NSAIDS are not risk factors.

    Clinical Stages

    • Stage IV: Behavioral abnormalities & personality changes → PFC
    • Stage III: Visuo-spatial disorientation → Angular gyrus
    • Stage II: Stage of Anomia: naming and comprehension abnormalities
    • Stage I: Korsakoff Amnestic Stage: episodic memory loss (difficulty in consolidating memory) → Hippocampus
    • Time dependant (memory) → Contextual (memory) → Autobiographic (memory)
    • Individuals with daily use of cognitive domains are protected from AD.

    Direct and Indirect Pathways

    • All input from cortex to Striatum.
    • All output leaves through GPi.
    • The only excitatory nucleus in BG: Subthalamus.
    • SN → Corpus striatum: Via D1: Activates direct pathway, Via D2: Inhibits indirect pathway
    • Degeneration of NS pathway (Parkinson's disease (PD)) → Inhibition of cortex
    • Akinesia, bradyphrenia (slowness of thoughts)

    BG Disorders

    • Movement Disorders:
      • Hyperkinetic
      • Paucity of movement (↓ amplitude, slowness) unrelated to weakness/spasticity
    • Idiopathic Parkinson's Disease: The most common neurodegenerative disease, majority of cases are sporadic, affects the elderly > 60 years.

    3. CADASIL

    • Cerebral autosomal dominant arteriopathy with small vessel ischemic changes, leukoencephalopathy.
    • Also known as CADASIL with stroke and ischemic leukoencephalopathy.
    • Most common inherited vascular dementia.
    • NOTCH 3 mutation.
    • Presentation: Aura + migraine + dementia + stroke.

    Normal Pressure Hydrocephalus (NPH)

    • Hydrocephalus:
      • Occlusive (Non-communicating): Aqueductal stenosis
      • Non-occlusive (Communicating): ↑ CSF production, ↓ CSF adsorption
    • Atrophy: Obtuse interventricular angle.
    • NPH: Adsorption defect > Outflow resistance of CSF → Ventriculomegaly, no atrophy
    • Symptoms progress over weeks to months.
    • Abnormalities in NPH:
      • Gait: Gait apraxia (magnetic foot)
      • Urinary: Incontinence
      • Dementia: Subcortical type.
    • Arrest in initiation of ambulation.
    • Gait instability with multiple falls.
    • Generalized slow movements.
    • CSF pressure: Normal (~20 ml/day produced)
    • Treatment: Rx: Shunting (Good prognosis if effective)
    • NPH Etiology:
      • Idiopathic (NPH)
      • Familial
      • Congenital
      • Acquired: Trauma, SAH

    Basal Ganglia (BG)

    • Nuclei:

      • Caudate nucleus (CN)
      • Putamen
      • Globus Pallidus (GP): GPI (Internal), GPe (External)
      • Subthalamic nucleus (STN)
      • Substantia nigra (SN)
      • Corpus Striatum
      • Lenticular Nucleus
      • Lesion: Hypokinetic movements: Chorea, Dystonia/Athetosis, Hemiballismus

    • Functions:

      • Complex motor activity
      • Initiation and scaling of movement
      • Cognitive part of motor activity
      • Activity set for movement (Planning, programming & initiation)

    • Premotor cortex and supplementary motor area also involved in planning and programming.

    Common Features

    • Young onset dementia
    • Rapidly progressive dementia
    • No immune response

    Types of Prion Diseases

    • Creutzfeldt-Jakob disease (CJD)
    • Familial fatal insomnia
    • Kuru
    • Gerstmann-Straussler-Scheinker disease

    CJD

    • No immune response/inflammation
    • Long incubation period
    • Pathology:
      • Spongiform changes in cerebral cortex (cortical ribboning)
      • Basal ganglia hyperintensities
    • Symptoms: Dementia + myoclonus

    Investigations

    • EEG: Low voltage background, high voltage sharp waves
    • CSF: Normal
    • Brain biopsy: To confirm diagnosis
    • RX: Flupirtine maleate (centrally acting non-opioid analgesic)

    Vascular Dementia

    • Types:
      • Large vessel disease (Post-CVA dementia)
      • Small vessel disease

    Large Vessel Disease

    • Most common infarct site: Bilateral posterior cerebral artery (PCA) lesion.
    • Criteria:
      • Dementia
      • Cerebrovascular disease: Multi-infarct state with "strategic territory" infarct (infarct in these areas leads to dementia)

    Dementia and Associated Proteins

    Dementia Protein
    Alzheimer's Αβ
    FTD Tau
    DLB a synuclein
    CJD Prpsc

    Lewy Bodies

    • Intraneuronal cytoplasmic inclusions.
    • Content: α-synuclein.
    • α-synuclein-opathies.
    • Characteristic changes 5–10 years before onset of disease:
      • REM sleep disorders
      • Autonomic nervous system symptoms:
        • Postural hypotension
        • Erectile dysfunction
        • Urinary abnormalities

    DLB Dementia vs Parkinson's Disease

    Feature DLB Dementia Parkinson's Disease
    Cognitive impairment Precedes/ within 1 year of motor manifestations. Predominantly rapidly progressive dementia. After 4–5 years of motor manifestations.
    Visual hallucinations & fluctuating alertness Precedes onset of motor symptoms. Common
    Tremors Uncommon Common
    Rigidity B/L axial rigidity (Rocket sign +) Common
    Gait abnormalities Common Asymmetrical
    Effect of antipsychotics Worsens symptoms (d/t dopamine receptor inhibition) Uncommon
    Effect of L-dopa No improvement of symptoms Improves symptoms
    Treatment None available L-dopa, deep brain stimulation
    • Falling backwards immediately after trying to get up from a chair is a key sign.
    • Parkinson's: α-synucleinopathy
    • Alzheimer's: "Tau"pathy
    • Degenerated cholinergic neurons in the nucleus basalis of Meynert
    • Anticholinergics: Rx of drug-induced parkinsonism.

    1.Binswanger Disease

    • Most important risk factor: Hypertension (HTN)
    • Lacunar infarct
    • Pathology:
      • Lipohyalinotic infarct
      • Involves penetrating vessels (Size: 30 - 300 μm)
    • MRI finding: White matter hyperintensities in elderly

    2.Cerebral Amyloid Angiopathy

    • Aβ40 protein deposition
    • Elderly
    • Microhemorrhages

    Relationship between Dementia and Stroke

    • Onset of dementia within 3 months of stroke
    • Stepwise decline in cognitive function

    Clinical Features (Similar to Vascular Parkinson's)

    Fibres Affected Manifestation
    Corticobulbar tract Pseudobulbar palsy Gait disturbances & falls Gait apraxia Magnetic/ignition foot phenomenon (feet "stuck" to floor)
    Corticospinal tract (Affects lower limb fibres close to the ventricle) Gait disturbances & falls Gait apraxia Magnetic/ignition foot phenomenon (feet "stuck" to floor)
    Fibres from paracentral lobule Urinary abnormalities Personality changes
    Corticostriate fibres (Fibres from PFC to striatum) Urinary abnormalities Personality changes

    Subcortical v/s Cortical Dementia

    Cortical Dementia

    • Alzheimer's
    • FTD (Frontotemporal dementia)

    Subcortical Dementia

    • DLB
    • PSP
    • NPH
    • Binswangers disease

    Affected Regions

    Feature Subcortical Dementia Cortical Dementia
    Affected regions Hippocampus, medial temporal lobe Basal ganglia; Thalamus; Subcortical white matter; Corticospinal fibres; Corticobulbar fibres

    Symptoms

    Symptom Subcortical Dementia (Severity) Cortical Dementia (Severity)
    Memory Impairment Severe (+) Less severe (-)
    Apraxia Superior parietal lobe lesion -
    Agnosia Association areas lesion -
    Acalculia Angular gyrus lesion -
    Language - -
    Visuo-spatial defect - -
    Personality Early dysphasia; more severe; indifferent -
    Mood Depressed (Subcortical > Cortical) -
    Coordination Normal Impaired
    Cognitive & motor speed Normal Slow
    Abnormal movements Rare Common
    Extrapyramidal symptom - -

    Prion Diseases

    • Prion: Infectious protein with no nucleic acid.
    • Genetic/Sporadic
    • Pathology: ß pleated PrPsc protein → Spongiform degeneration of cortex → Neuronal loss.

    Investigations

    • EEG: Normal/non-specific slowing
    • CSF analysis: $β$42 levels, hyperphosphorylated tau levels

    PET Scan

    • 10C
    • Detects hypometabolism
    • Amyloid fibrils

    MRI

    • Images show MRI scans for Alzheimer's disease and control patients.

    Treatment

    • Cholinesterase inhibitors (DOC): Donepezil, Rivastigmine, Galantamine
    • NMDA antagonist: memantine
    • Monoclonal antibody against $Aβ42$: Aducanumab
    • Tacrine: Obsolete (due to hepatotoxicity)

    PIB PET Scans

    • Images show PIB PET scans for Alzheimer's disease and control patients. The color scale indicates the level of amyloid deposition, with more intense colors indicating higher levels.

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    Description

    This quiz covers essential concepts related to various forms of dementia, including Frontotemporal Dementia and Alzheimer's disease. Test your knowledge on clinical features, genetic factors, and stages of dementia, as well as their associated pathologies. Perfect for students and professionals in neurology or psychology.

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