Medicine Marrow Pg No 577-586 (Neurology)
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Questions and Answers

Which of the following statements about the basal ganglia pathways is correct?

  • The indirect pathway is primarily activated through D1 receptors.
  • The subthalamus is an inhibitory nucleus in the basal ganglia.
  • The striatum receives excitatory input from the cortex. (correct)
  • All output from the cortex goes to GPi.
  • The nigrostriatal pathway activates the indirect pathway through D2 receptors.

    False

    What condition is characterized by degeneration of the nigrostriatal pathway?

    Parkinson's disease

    A common symptom of Parkinson's disease includes _____ of movement.

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

    What is the most common type of Frontotemporal Dementia?

    <p>Behavioral variant</p> Signup and view all the answers

    Match the following terms to their respective features:

    <p>Hyperkinetic = Paucity of movement D1 Receptors = Activates direct pathway Subthalamus = Only excitatory nucleus Bradyphrenia = Slowness of thoughts</p> Signup and view all the answers

    Diffuse Lewy Body Dementia is the most common cause of dementia.

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

    What is the age of onset for Frontotemporal Dementia?

    <p>50 - 60 years</p> Signup and view all the answers

    Involves the MAPT gene on chromosome ______.

    <p>17</p> Signup and view all the answers

    Match the following types of dementia with their characteristics:

    <p>Frontotemporal Dementia = 70% sporadic, young onset (50-60 years) Diffuse Lewy Body Dementia = 2nd most common cause of dementia Alzheimer's Disease = 90-95% sporadic Progressive Supranuclear Palsy = A tauopathy</p> Signup and view all the answers

    What is the main genetic mutation associated with CADASIL?

    <p>NOTCH 3</p> Signup and view all the answers

    Normal Pressure Hydrocephalus (NPH) is characterized by ventriculomegaly without atrophy.

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

    List one common clinical feature of Normal Pressure Hydrocephalus.

    <p>Gait apraxia or urinary incontinence or dementia</p> Signup and view all the answers

    The most common inherited vascular dementia is ______.

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

    Match the following features with their corresponding condition:

    <p>Aura + migraine + dementia + stroke = CADASIL Gait apraxia and urinary incontinence = NPH Occlusive Hydrocephalus = Aqueductal stenosis Idiopathic, familial, congenital, acquired = NPH Etiology</p> Signup and view all the answers

    Which of the following nuclei is NOT part of the Basal Ganglia?

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

    The Basal Ganglia are exclusively responsible for cognitive activities.

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

    Name one function of the Basal Ganglia.

    <p>Complex motor activity</p> Signup and view all the answers

    The _____ nucleus is known to have an internal and external section in the Basal Ganglia.

    <p>Globus Pallidus</p> Signup and view all the answers

    Match the following functions with the appropriate components of the Basal Ganglia:

    <p>Caudate nucleus = Cognitive part of motor activity Putamen = Complex motor activity Subthalamic nucleus = Involvement in movement regulation Substantia nigra = Dopamine production</p> Signup and view all the answers

    What is a key characteristic of cognitive impairment in Dementia with Lewy Bodies (DLB)?

    <p>Precedes or occurs within 1 year of motor manifestations</p> Signup and view all the answers

    In Parkinson's disease, visual hallucinations and fluctuating alertness are common symptoms.

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

    What substance is primarily found in Lewy Bodies?

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

    A common effect of antipsychotic treatment in Dementia with Lewy Bodies is that it _____ symptoms.

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

    Match the following symptoms with their corresponding condition:

    <p>Tremors = Parkinson's Disease Rapidly progressive dementia = DLB Dementia Uncommon visual hallucinations = DLB Dementia Bilateral axial rigidity = DLB Dementia</p> Signup and view all the answers

    What is the most significant risk factor for Binswanger Disease?

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

    Cerebral Amyloid Angiopathy is characterized by Aβ40 protein deposition and is commonly seen in the elderly.

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

    What type of infarct is associated with Binswanger Disease?

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

    Within 3 months of a stroke, the onset of _____ is often observed.

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

    Match the clinical features with the affected fibres in Binswanger Disease:

    <p>Corticobulbar tract = Pseudobulbar palsy and gait disturbances Corticospinal tract = Gait disturbances and falls Fibres from paracentral lobule = Urinary abnormalities and personality changes Corticostriate fibres = Urinary abnormalities and personality changes</p> Signup and view all the answers

    Which type of dementia is characterized by spongiform changes in the cerebral cortex?

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

    Rapidly progressive dementia is a common feature of prion diseases.

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

    What protein is associated with Creutzfeldt-Jakob disease?

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

    In large vessel disease, the most common infarct site is the __________ PCA lesion.

    <p>B/1</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) = PrPsc</p> Signup and view all the answers

    Which type of rigidity is characterized by both rigidity and tremor at the wrist in Parkinson's Disease?

    <p>Cogwheel rigidity</p> Signup and view all the answers

    Patients with Parkinson's Disease typically exhibit increased blinking.

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

    What gait pattern is described as narrow-based with short quick steps and freezing in between?

    <p>Festinant gait</p> Signup and view all the answers

    In Parkinson's Disease, the absence of the ______ sign on an MRI may indicate certain changes in the midbrain.

    <p>swallow tail</p> Signup and view all the answers

    Match the symptoms of Parkinson's Disease with their descriptions:

    <p>Bradykinesia = Slowness of movement Hypophonia = Reduced voice volume Hypomimia = Minimal facial expression Froment's sign = Activity-induced increase in rigidity</p> Signup and view all the answers

    Which type of dementia is characterized by severe memory impairment and normal cognitive & motor speed?

    <p>Subcortical dementia</p> Signup and view all the answers

    Cortical dementia is associated with highly impaired coordination.

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

    Name one example of a subcortical dementia.

    <p>Diffuse Lewy Body Dementia (DLB)</p> Signup and view all the answers

    In cortical dementia, the affected regions include the ______ ganglia.

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

    Match the following symptoms to their corresponding type of dementia:

    <p>Severe memory impairment = Subcortical dementia Impaired coordination = Cortical dementia Depressed mood = Subcortical dementia Slow cognitive speed = Cortical dementia</p> Signup and view all the answers

    Which of the following symptoms is less severe in cortical dementia compared to subcortical dementia?

    <p>Memory impairment</p> Signup and view all the answers

    Agnosia is a common symptom of both subcortical and cortical dementia.

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

    The primary pathology in prion diseases involves the formation of ______ proteins.

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

    What type of tremor is predominantly seen in Parkinson's disease?

    <p>At rest tremor</p> Signup and view all the answers

    Essential tremor is usually asymmetric in its distribution.

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

    What medication class is typically used to alleviate tremors in Parkinson's disease?

    <p>Dopamine agonists</p> Signup and view all the answers

    In essential tremor, writing is typically characterized as _____ and tremulous.

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

    Match the following characteristics to either Parkinson's disease tremor or essential tremor:

    <p>Asymmetric = Parkinson's Disease Tremor Stable or slowly progressive = Essential Tremor Body parts affected: hands, head, voice = Essential Tremor Present extrapyramidal signs = Parkinson's Disease Tremor</p> Signup and view all the answers

    Study Notes

    Frontotemporal Dementia (Pick's Disease)

    • Occurs in 70% of cases spontaneously
    • Onset typically occurs between 50-60 years old
    • Characterised by rapid progression
    • Pathology involves the MAPT gene on chromosome 17
    • Protein Tau becomes hyperphosphorylated
    • Other proteins associated with the disease are Fus
    • Bilateral atrophy of the prefrontal cortex
    • Most common type is Behavioural Variant
    • Symptoms include Loss of Social Embarrassment, Dysfunction of Social and Emotional Systems
    • Apathy, Overeating, Impaired Planning and Judgement also frequently occur
    • Memory is relatively preserved
    • Temporar lobe involvement results in Increased Oral Behavior
    • Hyperphosphorylated Tau is a characteristic of both Alzheimer's and Pick's disease

    Diffuse Lewy Body Dementia

    • Second most common cause of Dementia
    • Associated with Parkinson-Plus and Atypical Parkinson's Syndrome
    • Parkinson-Plus syndrome involves "Tau"pathies, specifically Progressive supranuclear palsy, Corticobasal degeneration
    • "a synuclein'-opathies like Multiple system atrophy and Diffuse Lewy Body dementia are also linked
    • Diagnosis may be related to the presence of certain proteins in the brain
    • Time Stamps of the video on this topic are 00:00:27 and 00:07:10

    Direct and Indirect Pathways

    • All input from the Cortex to the Striatum
    • All output goes through the Globus Pallidus Internal (GPi)
    • The Subthalamus is the only excitatory nucleus in the Basal Ganglia
    • The Nigrostriatal Pathway connects the Substantia Nigra (SN) to the Corpus Striatum
    • Via D1 receptors, the direct pathway is activated
    • The indirect pathway is inhibited via D2 receptors

    CADASIL

    • Cerebral autosomal dominant arteriopathy with small vessel ischemic changes, leukoencephalopathy
    • Also referred to as CADASIL with stroke and ischemic leukoencephalopathy
    • Most common inherited vascular dementia
    • Characterized by a NOTCH 3 mutation
    • Presentation includes aura + migraine + dementia + stroke

    Normal Pressure Hydrocephalus (NPH)

    Hydrocephalus

    • Occlusive (Non-communicating) Hydrocephalus is caused by Aqueductal Stenosis
    • Non-occlusive (Communicating) Hydrocephalus is caused by increased CSF production or decreased CSF absorption

    NPH

    • Obtuse interventricular angle denotes atrophy
    • Characterised by an adsorption defect and outflow resistance of CSF, leading to Ventriculomegaly
    • Atrophy does not occur
    • Symptoms progress over weeks to months
    • Gait apraxia (magnetic foot), urinary incontinence, and subcortical dementia are abnormalities commonly observed
    • Normal CSF pressure (~20 ml/day produced)
    • Treatment involves Shunting, with good prognosis if effective

    NPH Etiology

    • Most cases are idiopathic
    • Other etiologies include: Familial, Congenital, and Acquired (Trauma, SAH)

    Basal Ganglia (BG)

    Gross Anatomy

    • Includes the following nuclei:
      • Caudate nucleus (CN)
      • Putamen
      • Globus Pallidus (GP)
      • Subthalamic nucleus (STN)
      • Substantia nigra (SN)
      • Corpus Striatum
      • Lenticular Nucleus

    Physiology

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

    Common Features of Prion Diseases

    • Young onset dementia
    • Rapidly progressive dementia
    • No immune response
    • Examples include: Creutzfeldt-Jakob disease (CJD), Familial fatal insomnia, Kuru, Gerstmann-Straussler-Scheinker disease

    CJD

    • No immune response/inflammation
    • Long incubation period
    • Pathology reveals Spongiform changes in the cerebral cortex and Basal ganglia hyperintensities
    • Symptoms include dementia + myoclonus

    Investigations for CJD

    • EEG shows low voltage background, high voltage sharp wave activity
    • CSF is normal
    • Brain biopsy is required for diagnosis confirmation
    • Flupirtine maleate can be used as treatment (centrally acting non-opioid analgesic)

    Vascular Dementia

    • Large vessel disease (Post-CVA dementia) and small vessel disease
    • Large vessel disease, most common infarct site is Bilateral Posterior Cerebral Artery (PCA) lesion
    • Criteria include:
      • Dementia
      • Cerebrovascular disease, particularly multi-infarct state with "strategic territory" infarct (infarcts in areas that lead to dementia)

    Lewy Bodies

    • Intraneuronal cytoplasmic inclusions
    • Composed of alpha-synuclein
    • Alpha-synuclein-opathies

    DLB Dementia vs Parkinson's Disease

    • DLB Dementia
      • Cognitive impairment precedes/ within 1 year of motor manifestations.
      • Shows predominantly rapid progressive dementia.
      • Visual hallucinations and fluctuating alertness precede onset of motor symptoms
      • Tremors are uncommon
      • Bilateral axial rigidity is present (Rocket sign +)
      • Gait abnormalities are common
      • Antipsychotics worsen symptoms (due to dopamine receptor inhibition)
      • L-dopa does not improve symptoms
      • No specific treatment available
    • Parkinson's Disease
      • Cognitive impairment occurs after 4–5 years of motor manifestations
      • Visual hallucinations are common
      • Tremors are common
      • Rigidity is common
      • Gait abnormalities are common but are asymmetrical
      • Antipsychotics have uncommon effects
      • L-dopa improves symptoms
      • Treatment includes L-dopa, deep brain stimulation
    • Falling immediately backward after attempting to get up from a chair is characteristic of both conditions.

    Binswanger Disease

    • Most important risk factor is Hypertension
    • Characterized by Lacunar infarct
    • Pathology involves Lipohyalinotic infarct affecting penetrating vessels (size: 30 - 300 μm)
    • MRI shows White matter hyperintensities in elderly

    Cerebral Amyloid Angiopathy

    • Aβ40 protein deposition
    • Found in elderly individuals
    • Associated with 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)

    • Corticobulbar tract involvement causes Pseudobulbar palsy, gait disturbances & falls, gait apraxia and the Magnetic/ignition foot phenomenon (feet "stuck" to floor)
    • Corticospinal tract involvement (affects lower limb fibers close to the ventricle) causes Gait disturbances & falls, gait apraxia and the Magnetic/ignition foot phenomenon
    • Fibers from the paracentral lobule are associated with Urinary abnormalities and Personality changes
    • Corticostriate fibers (Fibres from PFC to striatum) are associated with Urinary abnormalities and Personality changes

    Subcortical v/s Cortical Dementia

    • Cortical Dementia
      • Includes Alzheimer's and Frontotemporal dementia (FTD)
    • Subcortical Dementia
      • Includes Diffuse Lewy Body Dementia (DLB), Progressive Supranuclear Palsy (PSP), Normal Pressure Hydrocephalus (NPH), and Binswanger 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
      • Prions are infectious proteins with no nucleic acid.
      • Can be genetic or sporadic
      • Pathology involves Beta-pleated PrPsc protein, leading to Spongiform degeneration of the cortex and Neuronal loss

    Parkinson's Disease

    Symptoms

    • Hypertonia
      • Two types: Cogwheel rigidity (rigidity + tremor) and lead pipe rigidity
      • Froment's sign is activity-induced increase in contralateral rigidity
    • Akinesia/Bradykinesia
      • Bradyphrenia, reduced arm swinging, flexion hypertonia, short quick steps with freezing in between, festinant gait, micrographia, hypomimia, hypophonia, reduced blinking
      • Glabellar tap sign is positive
    • Atypical PD
      • Bilateral axial rigidity, difficulty turning in bed/rising from a chair (wheelchair sign)
    • Non-Parkinson's lesions with hypokinesia, specifically Hypothyroidism
    • Postural Instability
      • Unsteadiness and falls
      • Pull test: Patient falls backward

    Investigation

    • Clinical diagnosis
    • MRI:
      • Hyperintensities bilaterally in grey matter surrounded by hypointense red nuclei and crural fibres at the midbrain level
      • Swallow tail sign is absent (Swallow tail sign is present due to SN)

    Additional Notes

    • PD patients can also experience sensory symptoms (pain, anosmia), neuropsychiatric symptoms (depression > dementia), and gastrointestinal symptoms (dysphagia)

    Comparison of Parkinson's Disease Tremor and Essential Tremor

    Characteristics Parkinson's Disease Tremor Essential Tremor
    Tremor At rest +/- reemergent tremor Postural tremor
    Frequency 4-6 Hz 5-12 Hz
    Distribution Asymmetric Mostly symmetric
    Body parts affected Hands ± legs Hands, head, voice
    Writing Small (micrographia) Large and tremulous
    Course Progressive Stable or slowly progressive
    Family history uncommon (1%) Present
    Extrapyramidal signs (bradykinesia, rigidity, and loss of postural reflex) Present Absent
    Relieving factors Levodopa, dopamine agonists, anticholinergics Alcohol, propranolol, primidone, topiramate, gabapentin, clonazepam
    Usual site for surgical treatment with deep brain stimulation Subthalamic nucleus or globus pallidus interna Ventral intermediate thalamus

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    Description

    This quiz covers key features of Frontotemporal Dementia (Pick's Disease) and Diffuse Lewy Body Dementia. Learn about their symptoms, pathologies, and genetic associations. Test your knowledge on dementia types and their progression.

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