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87 Cards in this Set
- Front
- Back
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What are the deficits in dementia? |
Decreased cognitive ability, memory, or function, with intact consciousness
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What are the top two most common cause of dementia in the elderly?
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1. Alzheimer Disease
2. Multi-infarct |
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What can increase your risk of getting Alzheimer Disease?
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- Down syndrome (Amyloid Precursor Protein - APP is on chr 21)
- Familial forms (10% of cases) |
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What are the types of familial Alzheimer Disease? What is affected?
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Early onset:
- Amyloid Precursor Protein (APP) - chr 21 - Presenilin-1 - chr 14 - Presenilin-2 - chr 1 Late onset: - ApoE4 - chr 19 |
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What can protect against Alzheimer Disease?
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ApoE2 (chr 19)
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What are the gross changes in Alzheimer Disease?
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- Widespread cortical atrophy
- Narrowing of gyri - Widening of sulci |
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What hormone changes occur in Alzheimer Disease?
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↓ ACh
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What are the histologic change associated with Alzheimer Disease?
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- Senile Plaques
- Neurofibrillary tangles |
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What are the contents and effect of a senile plaque?
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- Extracellular β-amyloid core
- May cause amyloid angiopathy → intracranial hemorrhage - Aβ (Amyloid-β) synthesized by cleaving Amyloid Precursor Protein (APP) - Associated with Alzheimer Disease |
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What are the contents and effect of a neurofibrillary tangle?
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- Intracellular hyperphosphorylated tau protein = insoluble cytoskeletal elements
- Tangles correlate with degree of dementia - Associated with Alzheimer Disease |
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What type of dementia is associated with aphasia, parkinsonian aspects, and a change in personality?
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Pick Disease (Frontotemporal Dementia)
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What are the gross changes in Pick Disease (Frontotemporal Dementia)?
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- Frontotemporal atrophy
- Spares parietal lobe and posterior 2/3 of superior temporal gyrus |
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What is the histologic finding in Pick Disease (Frontotemporal Dementia)?
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Pick bodies = spherical tau protein aggregates
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What type of dementia is associated with visual hallucinations followed by parkinsonian features?
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Lewy Body Dementia
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What defect is associated with Lewy Body Dementia?
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α-Synuclein defect
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What type of dementia is rapidly progressive (weeks to months) and causes myoclonus ("startle myoclonus")?
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Creutzfeldt-Jakob disease
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What is the cause and effect of Creutzfeldt-Jakob disease?
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- Prions (PrP-c → PrP-sc sheet [β-pleated sheet resistant to proteases])
- Causes a spongiform cortex - Dementia is rapidly progressive (weeks to months) and associated with myoclonus |
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What other diseases can cause dementia?
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- Multi-infarct (2nd most common cause of dementia in elderly)
- Syphilis - HIV - Vitamins B1, B3, or B12 deficiency - Wilson disease - NPH |
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What vitamin deficiencies can cause dementia?
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Vitamins B1, B3, or B12
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What causes Multiple Sclerosis?
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Auto-immune inflammation and demyelination of the CNS (brain and spinal cord)
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What are the common symptoms in a patient with Multiple Sclerosis?
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- Optic neuritis (sudden loss of vision)
- Internuclear Ophthalmoplegia - Hemiparesis - Hemisensory symptoms - Bladder / bowel incontinence |
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Who is most commonly affected by Multiple Sclerosis? Clinical course?
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- Most often women in 20s and 30s
- More common in whites - Relapsing and remitting course |
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What is the classic triad of symptoms in Multiple Sclerosis?
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SIN:
- Scanning speech - Intention tremor (also Incontinence and Internuclear ophthalmoplegia) - Nystagmus |
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What are the lab findings associated with Multiple Sclerosis?
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Increased protein (IgG) in CSF
*Oligoclonal bands are diagnostic |
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What is the best way to diagnose Multiple Sclerosis? What findings do you look for?
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MRI is the gold standard
- Periventricular plaques (areas of oligodendrocyte loss and reactive gliosis) with destruction of axons - Multiple white matter lesions separated in space and time |
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How do you treat Multiple Sclerosis?
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- β-interferon
- Immunosuppression - Natalizumab - Symptomatic treatment for neurogenic bladder (catheter, muscarinic antagonists) - Symptomatic treatment for spasticity (baclofen, GABA receptor agonist) - Symptomatic treatment for pain (opioids) |
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What drugs are given to patients with Multiple Sclerosis regardless of symptoms?
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- β-interferon
- Immunosuppression - Natalizumab |
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What drugs are given to patients with Multiple Sclerosis with neurogenic bladder problems?
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- Catheterization
- Muscarinic antagonists |
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What drugs are given to patients with Multiple Sclerosis with spasticity problems?
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- Baclofen
- GABA receptor agonist |
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What drugs are given to patients with Multiple Sclerosis with pain problems?
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Opioids
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What is the most common variant of Guillain-Barré Syndrome?
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Acute Inflammatory Demyelinating Polyradiculopathy
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What causes Acute Inflammatory Demyelinating Polyradiculopathy?
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Auto-immune condition that destroys Schwann cells → inflammation and demyelination of peripheral nerves and motor fibers
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What are the consequences of the demyelination of the peripheral nerves and motor fibers in Acute Inflammatory Demyelinating Polyradiculopathy?
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- Symmetric ascending muscle weakness and paralysis beginning in lower extremities
- Facial paralysis in 50% of cases - Autonomic function may be severely affected (eg, cardiac irregularities, hypertension, or hypotension) - Almost all survive, majority recover after weeks to months |
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What are the lab findings associated with Acute Inflammatory Demyelinating Polyradiculopathy?
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- ↑ CSF protein
- Normal cell count (albuminocytologic dissociation) - ↑ Protein → Papilledema |
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What is Acute Inflammatory Demyelinating Polyradiculopathy associated with / cause?
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Infections:
- Campylobacter jejuni - CMV Leads to auto-immune attack of peripheral myelin due to molecular mimicry, inoculations, and stress, but no definitive link to pathogens |
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How do you treat a patient with Acute Inflammatory Demyelinating Polyradiculopathy?
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- Respiratory support is critical until recovery
- Additional: plasmapheresis, IV immune globulins |
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What disease is associated with AIDS patients and destroys oligodendrocytes leading to demyelination of CNS? Cause?
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Progressive Multifocal Leukoencephalopathy
- Associated with reactivation of latent JC virus infection |
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How common is Progressive Multifocal Leukoencephalopathy in AIDS patients?
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2-4%
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What is the prognosis for Progressive Multifocal Leukoencephalopathy?
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- Rapidly progressive
- Usually fatal |
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What increases the risk of Progressive Multifocal Leukoencephalopathy?
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- ↑ risk with Natalizumab
- More common in AIDS patients |
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What disease causes multi-focal perivenular inflammation and demyelination after infection or vaccinations?
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Acute Disseminated Encephalomyelitis
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What is the cause of Acute Disseminated Encephalomyelitis?
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- Occurs after infection: commonly measles or VZV
- Or occurs after vaccine: rabies or smallpox |
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What are the consequences of Acute Disseminated Encephalomyelitis?
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Multifocal perivenular inflammation and demyelination
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What disease is due to an arylsulfatase A deficiency? What builds up?
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Metachromatic Leukodystrophy
- Build up of sulfatides |
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What causes build up of sulfatides in Metachromatic Leukodystrophy? What does this lead to?
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- Autosomal recessive deficiency of Arylsulfatase A
- Leads to impaired production of myelin sheath |
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What are the findings of a patient with Arylsulfatase A deficiency (Metachromatic Leukodystrophy)?
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- Build up of sulfatides → impaired production of myelin sheath
- Central and peripheral demyelination - Ataxia and dementia |
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What disease is related to defective production of proteins involved in the structure and function of peripheral nerves or myelin sheath?
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Charcot-Marie-Tooth disease
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What is wrong in Charcot-Marie-Tooth disease?
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Hereditary motor and sensory neuropathy (HMSN)
- Progressive hereditary disorder related to defective production of proteins involved in structure and function of peripheral nerves or myelin sheath - Typically autosomal dominant |
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What is Charcot-Marie-Tooth disease associated with?
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- Scoliosis
- Foot deformities (high or flat arches) |
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What disease is associated with a deficiency of galactocerebrosidase? What builds up?
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Krabbe Disease
- Build up of galactocerebroside and psychosine which destroy the myelin sheath |
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What are the clinical findings caused by a deficiency of galactocerebrosidase (Krabbe Disease)?
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- Peripheral neuropathy
- Developmental delay - Optic atrophy - Globoid cells |
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What disease disrupts metabolism of very-long-chain fatty acids? Cause?
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Adrenoleukodystrophy
- X-linked (typically affects males) |
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What are the clinical findings caused by a disruption of very-long-chain fatty acid metabolism / Adrenoleukodystrophy?
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- Excessive build-up in nervous system, adrenal gland, ante testes
- Progressive disease that can lead to long-term coma/death and adrenal gland crisis |
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What is characterized by synchronized, high-frequency neuronal firing?
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Seizures
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How can you categorize seizures?
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- Partial / focal vs generalized
- Simple vs complex |
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What is the difference between partial and generalized seizures?
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- Partial / focal: affects 1 area of brain (but can generalize)
- Generalized: diffuse |
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What is the most common location for a partial seizure?
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Medial temporal lobe
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What typically precedes a partial seizure?
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Seizure aura
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What are the types of partial seizures? How do they differ?
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- Simple Partial (consciousness intact): motor, sensory, autonomic, psychic
- Complex Partial (impaired consciousness) |
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What are the types of generalized seizures?
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- Absence (petit mal)
- Myoclonic - Tonic-clonic (grand mal) - Tonic - Atonic |
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What is epilepsy?
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Disorder of recurrent seizures (febrile seizures are not epilepsy)
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What is status epilepticus?
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Continuous seizure for >30 minutes or recurrent seizures without regaining consciousness between seizures for >30 minutes
(Medical emergency) |
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What kind of seizure is characterized by a blank stare and causes no postictal confusion?
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Absence (petit mal) - 3 Hz
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What kind of seizure is characterized by quick, repetitive jerks?
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Myoclonic (generalized seizure)
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What kind of seizure is characterized by alternating stiffening and movement?
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Tonic-Clonic (grand mal) generalized seizure
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What kind of seizure is characterized by stiffening only?
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Tonic generalized seizure
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What kind of seizure is characterized by "dropping" (falling to floor) and is commonly mistaken for fainting?
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Atonic generalized seizure
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What are the common causes of seizures in a child?
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- Genetic
- Infection (febrile) - Trauma - Congenital - Metabolic |
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What are the common causes of seizures in an adult?
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- Tumors
- Trauma - Stroke - Infection |
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What are the common causes of seizures in the elderly?
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- Stroke
- Tumor - Trauma - Metabolic - Infection |
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What causes a headache?
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Irritation of structures such as the dura, cranial nerves, or extracranial structures
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What are the types of headaches?
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- Cluster
- Tension - Migraine |
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How do the types of headaches differ in terms of localization?
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Unilateral:
- Cluster - Migraine Bilateral: - Tension |
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How do the types of headaches differ in terms of duration?
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- Cluster: 15 min - 3 hours (repetitive)
- Tension: >30 min (typically 4-6 hours); constant - Migraine: 4-72 hours |
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Which type of headache causes repetitive brief headaches with excruciating periorbital pain with lacrimation and rhinorrhea?
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Cluster Headache
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Which type of headache may induce Horner syndrome?
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Cluster Headache
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Which type of headache causes a steady pain without photophobia, phonophobia, or auras?
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Tension Headache
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Which type of headache causes a pulsating pain with nausea, photophobia, and/or phonophobia?
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Migraine
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Which type of headache is due to irritation on CN V, meninges, or blood vessels? What is released?
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Migraine
- Release of substance P, CGRP, and vasoactive peptides |
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What do Cluster Headaches cause? Treatment?
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- Repetitive brief headaches
- Excruciating periorbital pain with lacrimation and rhinorrhea - May induce Horner syndrome - More common in males Treatment: - Inhaled O2 - Sumatriptan |
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What do Tension Headaches cause? Treatment?
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- Steady pain
- No photophobia, phonophobia, or auras Treatment: - Analgesics, NSAIDs, or acetaminophen - Amitriptyline for chronic pain |
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What do Migraine Headaches cause? Treatment?
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- Pulsating pain with nausea, photophobia, or phonophobia
- May have "aura" - Due to irritation of CN V, meninges, or blood vessels (release of substance P, CGRP, and vasoactive peptides) Treatment: - Abortive therapies (eg, triptans or NSAIDs) - Prophylactic therapies (eg, propranolol, topiramate, CCBs, or amitriptyline |
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What mnemonic helps you remember characteristics of migraine headaches?
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POUND
- Pulsatile - One day duration - Unilateral - Nausea - Disabling |
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What are the abortive therapies used for migraine?
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- Triptans (eg, Sumatriptan)
- NSAIDs |
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What are the prophylactic therapies used for migraine?
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- Propranolol
- Topiramate - Calcium channel blockers - Amitriptyline |
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What are some other causes of head pain (not a headache)?
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- Subarachnoid hemorrhage ("worst headache of my life")
- Meningitis - Hydrocephalus - Neoplasia - Arteritis |
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How can you distinguish a cluster headache from trigeminal neuralgia?
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Depends on duration:
- Cluster headache: 15 min - 3 hours (repetitive) - Trigeminal neuralgia: repetitive shooting pain in distribution of CN V that lasts typically for <1 minute |
