Neurology and Dementia Quiz

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Questions and Answers

What is the most common cause of dementia?

  • Lewy body dementia
  • Alzheimer's disease (correct)
  • Multi-infarct dementia
  • Pick's disease

What type of seizure is characterized by a sudden impairment of consciousness with no change in body or motor tone, lasting a few seconds?

  • Myoclonic seizure
  • Tonic-clonic seizure
  • Complex partial seizure
  • Absence seizure (correct)

What is the hallmark of Juvenile Myoclonic Epilepsy?

  • Abnormal motor activity
  • Epigastric rising sensation
  • Myoclonic jerks on awakening from sleep (correct)
  • Sudden impairment of consciousness

Which of the following is NOT a risk factor for Alzheimer's disease?

<p>Regular exercise (D)</p> Signup and view all the answers

What is the characteristic EEG finding in absence seizures?

<p>Spike and wave activity at 2.5-5 Hertz (B)</p> Signup and view all the answers

What is the most common site for seizures?

<p>Temporal lobe (D)</p> Signup and view all the answers

What is the term for the warning period before a major seizure?

<p>Aura (D)</p> Signup and view all the answers

Which of the following is NOT a common symptom of Alzheimer's disease?

<p>Sudden onset of confusion (B)</p> Signup and view all the answers

What is the difference between a simple partial seizure and a complex partial seizure?

<p>Simple partial seizures have no alteration in consciousness, while complex partial seizures involve an alteration in consciousness. (B)</p> Signup and view all the answers

Which of the following is NOT a common cause of a provoked seizure?

<p>High blood pressure (D)</p> Signup and view all the answers

Which type of medication is used as a first-line treatment for absence seizures?

<p>Ethosuximide (B)</p> Signup and view all the answers

What is the name of the protein found in Lewy bodies?

<p>Alpha-synuclein (C)</p> Signup and view all the answers

What is the term for a post-seizure period of brain recovery characterized by confusion, lack of alertness, and possible focal neurological deficits?

<p>Postictal state (A)</p> Signup and view all the answers

Which of the following is NOT a common type of generalized seizure?

<p>Jacksonian seizure (C)</p> Signup and view all the answers

What is the term for the enlargement of brain ventricles due to cortical atrophy?

<p>Hydrocephalus ex vacuo (B)</p> Signup and view all the answers

What is the primary role of acetylcholinesterase inhibitors, such as Donepezil, in the treatment of Alzheimer's disease?

<p>Increase levels of acetylcholine in the brain (A)</p> Signup and view all the answers

Which of these neurologic conditions is NOT caused by an autoimmune phenomenon?

<p>Progressive multifocal leukoencephalopathy (PML) (A)</p> Signup and view all the answers

What differentiates Pick's disease from Alzheimer's disease?

<p>Pick's disease involves spherical tau proteins called Pick bodies, unlike the tangles found in Alzheimer's disease (C)</p> Signup and view all the answers

What is the characteristic finding on MRI for a patient with multiple sclerosis?

<p>Periventricular plaques (D)</p> Signup and view all the answers

Which of the following is a classic feature of Creutzfeldt-Jakob disease?

<p>Startle myoclonus (D)</p> Signup and view all the answers

In the context of multiple sclerosis, what is the term used to describe the process where immune cells react to myelin antigens, leading to its destruction?

<p>Autoimmune demyelination (C)</p> Signup and view all the answers

Which of the following is NOT a treatment option for multiple sclerosis?

<p>Plasmapheresis (B)</p> Signup and view all the answers

Which of these is the classic agent that often triggers Guillain-Barre syndrome?

<p>Campylobacter jejuni (C)</p> Signup and view all the answers

What kind of cells are destroyed in Guillain-Barre syndrome?

<p>Schwann cells (B)</p> Signup and view all the answers

What is the characteristic CSF finding in patients with Guillain-Barre syndrome?

<p>Elevated protein level with normal cell count (B)</p> Signup and view all the answers

Which of the following is considered a classic neurologic manifestation of multiple sclerosis?

<p>Optic neuritis (C)</p> Signup and view all the answers

Which of the following is characteristically seen in the brains of patients with Creutzfeldt-Jakob disease?

<p>Intracellular vacuoles and PrPSC prions (C)</p> Signup and view all the answers

A patient presents with a sudden onset of ascending muscle weakness, starting in the legs and spreading to other areas. This is accompanied by mild sensory deficits, but the patient primarily exhibits a lack of strength. Which of the following conditions is most likely to be the cause?

<p>Guillain-Barre Syndrome (A)</p> Signup and view all the answers

What is the most common histopathological finding in the brain of a patient with postinfectious encephalomyelitis?

<p>Perivenous infiltration of lymphocytes, neutrophils, and other inflammatory cells (C)</p> Signup and view all the answers

Which of the following is a classic neurological manifestation of Multiple Sclerosis?

<p>Optic neuritis (D)</p> Signup and view all the answers

Which of the following is a characteristic clinical feature of Creutzfeldt-Jakob disease?

<p>Progressive and rapid dementia (C)</p> Signup and view all the answers

Which of the following is a common treatment option for Multiple Sclerosis?

<p>Interferon therapy (B)</p> Signup and view all the answers

In which of the following neurological conditions is a brain biopsy considered the gold standard for diagnosis?

<p>Creutzfeldt-Jakob disease (C)</p> Signup and view all the answers

Which of the following neurological conditions is typically triggered by a viral infection?

<p>Progressive Multifocal Leukoencephalopathy (C)</p> Signup and view all the answers

Which of the following is characterized by a reactivation of a latent virus in individuals with a compromised immune system?

<p>Progressive Multifocal Leukoencephalopathy (D)</p> Signup and view all the answers

Which of the following conditions is a rare, genetic disorder that affects the peripheral nervous system?

<p>Charcot-Marie-Tooth disease (B)</p> Signup and view all the answers

Flashcards

Pick disease

A rare cause of dementia affecting frontal and temporal lobes, characterized by personality changes and aphasia.

Prion diseases

Diseases caused by PrPSC prions that lead to spongiform encephalopathy with intracellular vacuoles.

Classic features of prion disease

Include rapidly progressive dementia, ataxia, startle myoclonus, and spike-wave complexes on EEG.

Multiple Sclerosis (MS)

A CNS demyelination disease primarily affecting young white women with relapsing-remitting symptoms.

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Optic neuritis

A common symptom of MS characterized by pain and loss of vision due to demyelination of the optic nerve.

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MRI in MS

Gold standard imaging for detecting periventricular plaques characteristic of multiple sclerosis.

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Guillain-Barre syndrome

An acute demyelinating disease causing ascending muscle weakness that may lead to respiratory failure.

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JC virus

A virus that reactivates in immunocompromised patients, causing progressive multifocal leukoencephalopathy (PML).

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CSF findings in demyelinating diseases

Increased protein levels and normal cell count are indicative of diseases like Guillain-Barre.

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Hereditary motor and sensory neuropathy (HMSN)

Progressive genetic nerve disorder causing muscle wasting and foot deformities, usually starting in childhood.

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Seizure

Sudden alteration in behavior due to transient brain pathology.

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Provoked seizure

A seizure often triggered by factors like fever, drugs, or lack of sleep.

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Epilepsy

Multiple unprovoked seizures occurring over time.

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Electroencephalogram (EEG)

Test that records voltage changes in the brain.

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Partial seizure

A seizure that affects one discrete part of the brain.

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Generalized seizure

A seizure that affects the entire brain.

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Aura

Warning signs before a major seizure, stemming from simple partial seizures.

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Hypothalamic dysfunction

Commonly seen in temporal lobe seizures causing unique sensations.

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Alzheimer’s disease

Most common dementia, leading to cognitive decline due to cortical degeneration.

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Amyloid plaques

Extracellular deposits found in Alzheimer’s disease that contribute to neuronal loss.

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Risk factors for Alzheimer’s

Age, genetics, family history, and lifestyle influences.

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Memantine

An NMDA receptor blocker used to treat Alzheimer's symptoms.

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Vascular dementia

Second most common dementia due to multiple strokes.

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Lewy body dementia

Characterized by dementia symptoms, Parkinson’s symptoms, and hallucinations.

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Post-ictal state

The recovery period after a seizure characterized by confusion and fatigue.

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Frontal lobe symptoms

Personality and behavior changes due to frontal lobe damage.

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Temporal lobe symptoms

Aphasia and language difficulties resulting from temporal lobe issues.

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Pick bodies

Spherical tau proteins found in certain dementia cases, unlike tangles in Alzheimer's.

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Prion diseases symptoms

Rapid dementia, ataxia, and startle myoclonus with spike-wave complexes seen on EEG.

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Optic neuritis symptoms

Pain and vision loss due to demyelination of the optic nerve.

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Acute inflammatory demyelinating radiculopathy

Characterized by rapid onset muscle weakness caused by immune destruction of Schwann cells.

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Multiple sclerosis pathology

Involves lymphocytic reaction to myelin, leading to diverse neurological symptoms.

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Diagnostic techniques in demyelinating diseases

MRI for plaques and CSF findings like high protein and oligoclonal bands.

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Transient brain pathology

Sudden changes in brain function causing behavioral changes.

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Loss of consciousness

A state where a person is not aware of their surroundings.

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Abnormal motor activity

Unusual movements or muscle control issues during seizures.

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Tonic-clonic seizure

A severe seizure involving muscle stiffness followed by jerking movements.

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Juvenile myoclonic epilepsy

A genetic form of epilepsy that often starts in childhood.

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Common seizure triggers

Factors like fever, drugs, and sleep deprivation that can provoke seizures.

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Aura in seizures

Warning signs experienced before a major seizure, linked to simple partial seizures.

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Mesial temporal sclerosis

Neuronal loss in the hippocampus, often visible in epilepsy diagnoses.

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EEG (Electroencephalogram) patterns

Characteristic voltage changes recorded from the brain during seizures.

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Alzheimer's disease symptoms

Progressive cognitive decline primarily affecting elderly and marked by memory loss.

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Neurofibrillary tangles

Twisted fibers of protein found in neurons, significant in Alzheimer's.

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Cognitive decline warning signs

Early signs like forgetting recent events or confusion in familiar surroundings.

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Risk factors for dementia

Factors like age, obesity, and family history increase dementia risk.

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NMDA receptor blockers

Medications like Memantine that help manage Alzheimer's symptoms.

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Study Notes

Seizure Overview

  • Seizures are sudden alterations in behavior caused by transient brain pathology.
  • Symptoms vary, ranging from mild (loss of awareness) to severe (tonic-clonic).
  • Some seizures are "provoked" (e.g., fever, lack of sleep, drugs, alcohol, hypoglycemia).
  • Others are unprovoked, indicating more serious issues (e.g., tumors, strokes).
  • Epilepsy is a medical condition defined as multiple, unprovoked seizures.

Seizure Causes by Age Group

  • Children: Genetic, fever, trauma, congenital, metabolic, or infection. Juvenile myoclonic epilepsy, hyponatremia, hypernatremia, hypomagnesemia, and hypocalcemia are examples.
  • Adults: Trauma, stroke, infection, or metabolic issues are common causes.
  • Elderly: Stroke, tumors, metabolic issues, or infection are common causes.

Seizure Workup

  • Bloodwork is often part of the evaluation; EKG for possible cardiac syncope and EEG to evaluate brain activity are also used.
  • Brain imaging (CT or MRI) can pinpoint the source of the seizure.
  • Lumbar puncture (LP) is sometimes necessary.

EEG (Electroencephalogram)

  • Records voltage changes in the brain.
  • Different leads (e.g., frontal, parietal, occipital) capture electrical activity from various brain regions.
  • Characteristic patterns can reveal the presence of seizures.
  • Electroencephalogram is typically normal in dementia but is abnormal with delirium.

Seizure Types

  • Partial Seizures: Affect a single area of the brain.
  • Simple partial: no impaired consciousness.
  • Complex partial: impaired consciousness.
  • Generalized Seizures: Affect all areas of the brain.
  • Absence (petit mal): brief loss of awareness
  • Tonic-clonic (grand mal): convulsions
  • Atonic (drop seizure): loss of muscle tone
  • Myotonic: muscle spasms

Psychic Symptoms

  • Possible symptoms experienced during partial seizures; higher cortical areas are affected.
  • Dysphasia (language problems), feelings of familiarity (déjà vu), distortions of time, fear, or hallucinations.

Autonomic Symptoms

  • Epigastric "rising" sensation, common with medial temporal lobe epilepsy; sweating, piloerection, or pupillary changes.

Auras

  • Warnings before major seizures, which are often simple or partial seizures.
  • The aura's symptoms often correlate with the area of the brain affected (e.g., flashing lights in occipital lobe).
  • Often not severe enough to disrupt consciousness.

Post-Ictal State

  • Period after a seizure, where the brain recovers to its normal state
  • Possible symptoms: confusion, lack of alertness, which typically resolve in minutes to hours.
  • Focal neurological deficits may emerge.

Partial Seizures (continued)

  • Temporal lobe is the most common seizure location.
  • Mesial temporal sclerosis (also called hippocampal sclerosis) is common. It is identified neurologically due to neuronal loss in the hippocampus.
  • Often bilateral, but one side may be more affected.
  • Can be diagnosed with MRI scans.

Juvenile Myoclonic Epilepsy

  • Absence, myoclonic, and grand mal seizures common in children.
  • Absence seizures first (around 5 years), followed by myoclonic (around 15 years) eventually progressing to grand mal seizures, though often appearing together.
  • Triggered by sudden awakening from sleep.
  • Characterized by shock-like, irregular movements in both arms.

Childhood Absence Epilepsy

  • Sudden loss of consciousness is the most prominent symptom.
  • No significant change in body/motor tone is experienced.
  • The occurrence is short-lived—lasting a few seconds typically—and episodic.
  • Usually stops at puberty.
  • Classic EEG finding; 2.5–5 Hertz spike-wave activity superimposed on normal EEG.
  • No post-ictal confusion is observed.
  • Ethosuximide is typically the first-line treatment but different from other epilepsy types.

Febrile Seizures

  • Common in children under 5 years old (2-4% prevalence).
  • Characterized by losing consciousness and jerking movements triggered by fever.
  • Children at higher risk for more febrile seizures.
  • Generally have a positive prognosis.
  • Not considered a type of epilepsy; considered an occurrence due to an external trigger.

Eclampsia

  • Pregnancy-related condition.
  • Occurs from 20 weeks to 6 weeks post-partum.
  • Preeclampsia (high blood pressure, proteinuria, and edema) is an indicator.
  • Eclampsia features preeclampsia symptoms plus seizures.
  • Treated with magnesium sulfate (MgSO4).

Seizure Treatment Principles

  • Breaking seizures: Status epilepticus (continuous seizure for more than 30 minutes), or repetitive seizures within 30 minutes requires immediate medical attention.
    • Benzodiazepines (e.g., Lorazepam) are the first-line treatment.
    • Phenytoin/fosphenytoin is frequently used to prevent reoccurrence.
    • Phenobarbital can be used if seizures continue. Often require general anesthesia and intubation.
  • Preventing seizures: Medication is administered to lessen the likelihood of recurrent seizures. Different classes of drugs are classified according to the mechanism they affect, including, sodium channel blockers, and GABA activators, along with other mechanisms.

Niche Drugs

  • Status epilepticus: Most commonly benzodiazepines.
  • Absence seizures: Ethosuximide.

Teratogenicity

  • All antiepileptic drugs (AEDs) pose a risk during pregnancy.
  • Valproic acid is the most teratogenic AED.
  • Neural tube defects have a high risk (1-3 %).

Carbamazepine

  • Inactivates sodium channels.
  • Effective for partial and generalized seizures.
  • Associated with many side effects(e.g., diplopia, ataxia, low blood counts, agranulocytosis, aplastic anemia, Stevens-Johnson syndrome).
  • Blood levels should be monitored; can cause bone marrow suppression and liver toxicity.

Ethosuximide

  • Blocks thalamic T-type calcium channels.
  • Common choice for childhood absence seizures.
  • Side effects include nausea, vomiting, sleep disruption, fatigue, and hyperactivity. May cause Stevens Johnson Syndrome.

Phenobarbital

  • Barbiturate, binding to GABA receptors, increasing channel opening duration.
  • Associated with less firing and less myocardial/respiratory depression compared to other treatment.
  • Less side effects but may have excessive CNS depression if combined with alcohol. Caution in use in patients with porphyria. Induces the P450 enzyme system.

Cytochrome P450

  • Important intracellular enzymes metabolizing many drugs.
  • Inhibition of CYP450 leads to increased drug levels, while induction leads to decreased levels.
  • AEDs can induce CYP450. Inhibitors of CYP450 can be dangerous as increased drug levels cause several complications.

P450 Drugs (examples)

  • Inducers: Chronic alcohol, rifampin, phenobarbital, carbamazepine, and griseofulvin and phenytoin.
  • Inhibitors: Isoniazid, erythromycin, cimetidine, azoles, grapefruit juice, and ritonavir.

Phenytoin

  • Inactivates sodium channels.
  • Useful for tonic-clonic seizures.
  • Can lead to gingival hyperplasia, hair growth and rash.
  • Folic acid supplementation and folic acid depletion are sometimes needed due to its side effects.
  • Long-term use is associated with nystagmus, diplopia, and ataxia.
  • Is considered teratogenic, and blood levels are important to follow. Dose-dependent hepatic metabolism is also relevant.

Valproic Acid

  • Sodium and GABA effects.
  • Often a mood stabilizer for bipolar disorder or acute mania.
  • Not recommended for women who are pregnant; may associate with spina bifida.
  • Hepatotoxicity and need for liver function monitoring (LFTS).
  • Associated with tremor and weight gain.

Levetiracetam

  • Exact mechanism unknown.
  • Effective for many types of seizures.
  • Blood levels can be monitored during treatment.
  • Well tolerated; few important/serious side effects are noted.

Lamotrigine

  • Sodium channel drug that can cause Stevens-Johnson Syndrome or SJS, especially in children.
  • Discontinuation if rash presents.
  • Affects calcium channels and has side effects such as sedation and ataxia.

Topiramate

  • Affects both sodium and GABA effects. Causes mental dulling, sedation, reduced weight and kidney stones.
  • Leads to more calcium in the urine, may increase the risk of kidney stones, and requires hydration to lessen the risk.

Primidone

  • Mechanism of action is less understood compared to other drugs.
  • Metabolized to phenobarbital.
  • Can also be used for essential tremor.

Neuroembryology and other sections remain unchanged.

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