Mayo Clinic 2 - 33 - Nondegenerative Dementias And Encephalopathies

Front 1

What is subcortical dementia?

Back 1

Dmentia primarily due to damage to the frontal subcortical connections

Front 2

Typical clinical features of subcortical dementia? (6)

Back 2

1. Inattention

2. Bradyphrenia

3. Executive dysfunction (difficulties planning and sequencing tasks)

4. Apathy

5. Psychomotor slowing and mood disorders

Front 3

Cognitive disorders related to cerebrovascular disease consist of 3 major subtypes, what are they?

Back 3

1. Vascular cognitive impairment but not dementia (not yet interfering with day-to-day activities)

2. Vascular dementia

3. Mixed vascular dementia and primary neurodegenerative dementia (usually Alzheimer disease)

Front 4

What is the risk of poststroke dementia after a clinical stroke?

Back 4

20%

Front 5

How many % of dementia cases are vascular dementia?

Back 5

Up to 20%

Front 6

Which is the most common criteria for the diagnosis of vascular diagnosis?

Back 6

National Institute of Neurological Disorders and Stroke-ASsociation Internationale Pour la Recherch e et l'Enseignment en Neuroscience

Front 7

Clinical features of Binswanger disease (8)

Back 7

1. Early gait apraxia and parkinsonian gait

2. Frequent falls

3. Focal motor deficits and pyramidal signs on examination (extensor plantar responses and exaggerated deep tendon reflexes)

4. Urinary urgency and incontinence

5. Apathy and psychomotor slowing

6. Attention and concentration difficulties

7. Frontal release signs and milder memory deficits than those that occur with other dementias

8. Executive dysfunction

Front 8

What is Binswanger disease?

Back 8

The classic syndrome of subcortical dementia from vascular disease

Front 9

Causes of vascular dementia (6)

Back 9

1. Hypoperfusion

2. .Hypoxic-ischemic injury

3. Large-vessel injury

4. Small-vessel injury

5. Coagulation disorders

6. Others (superficial siderosis, A beta-related vasculitis)

Front 10

Large-vessel disease that may be a cause of vascular dementia?

Back 10

Vasculitis, either granulomatous or systemic

Front 11

Small-vessel disease that may be a cause of vascular dementia? (4)

Back 11

1. Binswanger disease

2. Multiple lacunar infarctions

3. CADASIL

4. Spatz-Lindenberg disease

Front 12

Typical location of a stroke that results in apathy and psychomotor slowing?

Back 12

Dorsolateral caudate nucleus; recurrent artery of Huebner infarct

Front 13

Typical location of a stroke causing psychomotor hyperactivity, disinhibition, and impulsiveness?

Back 13

Ventromedial caudate nucleus; recurrent artery of Huebner

Front 14

Typical location of a stroke causing anterograde and retrograde amnesia?

Back 14

Hippocampi; often from hypoperfusion injury, typically bilateral

Front 15

Typical location of a stroke causing altered level of consciousness, lack of spontaneous behaviour, and vertical gaze palsy?

Back 15

Paramedian thalamus, Artery of Percheron; artery arisses at basilar bifurcation and supplies paramedian thalamus and rostral midbrain

Front 16

Typical location of a stroke resulting in acute delirium?

Back 16

Right parietal lobe

Front 17

Typical location of a stroke causing acalculia, agraphia, left-right dissociation and finger agnosisa?

Back 17

Dominant angular and supramarginal gyrus, Gerstmann syndrome; located in parietal lobe

Front 18

Typical location of a stroke causing optic ataxia, oculomotor apraxia, and simultanagnosia?

Back 18

Bilateral parieto-occipital lesions, Balint syndrome; often caused by bilateral watershed hypoperfusion infarcts

Front 19

Typical location of a stroke causing left-hand apraxia?

Back 19

Anterior corpus callosum

Front 20

Typical location of a stroke causing pseudobulbar palsy?

Back 20

Bilateral internal capsule infarcts

Front 21

Typical location in a stroke causing depression?

Back 21

Left frontal lobe

Front 22

Typical location in a stroke causing OCD?

Back 22

Posterior right frontal lobe

Front 23

MRI findings in vascular dementia?

Back 23

Extensive white matter T2-signal hyperintensities known as leukoaraiosis, which is particularly prominent in Binswanger disease

Front 24

MRI findings in HIV-associated neurocognitive disorder

Back 24

Global atrophy and T2-signal hyperintensities in the periventricular and deep white matter

Front 25

Time from infection to onset of neurosyphilis?

Back 25

3 to 40 years after infection with T.pallidum

Front 26

How does neurosyphilis present?

Back 26

With a frontal dysexecutive syndrome with inattention and executive dysfunction

Front 27

Autoimmune or paraneoplastic cognitive disorders can be broadly divided into two categories. What are they?

Back 27

1. Infrequently associated with cancer, which are termed autoimmune encephalopathies or dementias

2. Those associated with cancer, which are often termed paraneoplastic encephalopathies

Front 28

What is probably the most common cause of autoimmune encephalopathy?

Back 28

The voltage-gated potassium-channel complex autoantibody

Front 29

How does the voltage-gated potassium-channel complex antibody work?

Back 29

They bind to 2 proteins that assocciate with the channel

Front 30

How does autoimmune encephalitis usually present? (4)

Back 30

1. Subacute cognitive decline characterized by fluctuations

2. Frequently accompanied by tremor or myoclonus

3. Seizures may also coexist

4. Acute presentations occur as a classic limbic encephalitis

Front 31

When a paraneoplastic encephalopathy is under consideration, what else could be considered?

Back 31

Body CT or PET; neuron-specific autoantibodies may rule out paraneoplastic encephalopathy

Front 32

Treatment of autoimmune encephalopathy?

Back 32

High-dose i.v. methylprednisolone once daily for 5 days is recommended, and most patients improve within a few weeks

Front 33

NMDA encephalitis is related to which cancer?

Back 33

TEratoma, usually ovvarian

Front 34

Cognitive impairment is an underrecognized feature of MS. It affects approximately how many % of the patients?

Back 34

60%, although frank dementia occurs in less than 5%

Front 35

Thymomas are associated with which neural autoantibodies? (5)

Back 35

1. CRMP-5IgG

2. GAD65

3. Ganglionic acetylcholine receptor autoantibody

4. Glycine receptor antibodies

5. Caspr-2 (VGKC-complex)

Front 36

AMPA neural autoatibody is seen in which cancers? (3)

Back 36

1. Thymic tumors

2. Ling carcinomas

3. Breast carcinomas

Front 37

Thyroid peroxidase autoantibodies have been associated with what?

Back 37

Something that is termed Hashimoto encephalopathy or steroid-responsive encephalopathy associated with thyroid autoimmunity

Front 38

Supporting features of autoimmune encephalopathies

Back 38

1. Evidence of an inflammatory response on CSF examination

2. MRI evidence of T2-signal abnormalities in the mesial temporal lobe

Front 39

Back 39

MRI findings in autoimmune voltage-gated potassium channel complex-associated encephalopathy. T2-signal hyperintensity is greater in the left than the right mesial temporal lobes (white arrows)

Front 40

How long must an alcoholic patient be sober before assessment of dementia?

Back 40

At least 2 months, and treatment with alcohol cessation may result in some improvement

Front 41

Presentation of alcohol-related dementia (4)

Back 41

1. Frontal-dysexeccutive disorder with poor working memory

2. Inattention

3. Impaired judgement

4. Poor verbal fluency

Front 42

Triad of Wernicke-Korsakoff syndrome

Back 42

1. Global confusion

2. Truncal ataxia

3. Ophthalmoplegia or nystagmus

Front 43

Back 43

Brain with Marchiafava-Bignami disease. Note discoloration of the central portion of corpus callosum (arrow). Microscopic examination would show demyelination and necrosis

Front 44

Korsakoff syndrom consists of

Back 44

1. Anterograde amnesia and retrograde amnesia; recent memory is usually affected more than remote memory

2. Often confabulate early in the course

Front 45

This disease was initially described in drinkers of Italian red wine, but it has since been described in others with chronic alcoholism associated with other alcoholic beverages

Back 45

Marchiafavia-Bignami disease

Front 46

Hallmark finding in Marchiafava-Bignami disease is

Back 46

demyelination in the corpus callosum, usually involving the central regions

Front 47

MRI findings in Marchiafava-Bignami disease?

Back 47

Callosal enhancement early on and T2-signal changes and atrophy in the later stages

Front 48

Patients with a B12-deficiency may, in addition to dementia, present with which features? (4)

Back 48

1. Neuropsychiatric symptoms

2. Coexisting optic atrophy

3. Peripheral neuropathy

4. Subacute combined degeneration of the spinal cord

Front 49

The three ds of Pellagra?

Back 49

Diarrhea, dermatitis, and dementia, which at times may be rapidly progressive, mimicking CJD

Front 50

Inherited metabolic diseases that are associated with dementia? (4)

Back 50

1. Mitochondrial disorders

2. Inherited leukodystrophies

3. CADASIL

4. Lysosomal storage disorders (e.g., Neimann-Pick disease)

Front 51

One inherited disorder that deserves mention

Back 51

Adult polyglucosan body disease, beccause its presentation is often late in adulthood with an onset between the firth and seventh decades of life

Front 52

Presenting features of adult polyglucosan body disease (4)

Back 52

1. Dementia

2. Peripheral neuropathy

3. Neurogenic bladder

4. UMN signs

Front 53

Repetitive head trauma increases the risk of (3)

Back 53

1. Alzheimer disease

2. Chronic traumatic encephalopathy

3. Perhaps other neurodegenerative diseases

Front 54

Clinical features of postconcussive syndrome

Back 54

1. Inattention

2. Impaired working memory

3. Psychomotor slowing

4. Dizziness

5. Posttraumatic headache and psychiatric symptoms

Front 55

Which brain regions are most commonly affted by cerebral contusions?

Back 55

The frontotemporal regions

Front 56

Chronic traumatic encephalopathy is also called

Back 56

Dementia puilistica

Front 57

Unilateral temporal lobe injury may cause

Back 57

Learning and memory troubles

Front 58

Bilateral anterior temporal lobe lesions may cause

Back 58

Klüver-Bucy syndrome (hyperorality, hypersexuality, hypermetamorphosis, and docility)

Front 59

Underlying mechanism of chronic traumatic encephalopathy?

Back 59

Newer studies have shown that chronic traumatic encephalopathy is often associated with underlying tau abnormality and have suggested it be considered a progressive tauopathy

Front 60

Which MRI sequences may be helpful in the diagnosis of chronic traumatic encephalopathy?

Back 60

MRI using gradient echo sequences or susceptibility-weighted imaging, which is sensitive to hemosiderin deposition and may be helpful

Front 61

What are Lundberg waves?

Back 61

Intracranial pressure waveform

Front 62

Clinical presentation of normal pressure hydrocephalus?

Back 62

Gait disturbance (magnetic gait), urinary incontinence, and dementia

Front 63

The dementia in NPH is thought to be due to?

Back 63

Hydrocephalus impinging on periventricular frontostriatal projections

Front 64

MRI findings in NPH

Back 64

1. Ventriculomegaly out of proportion to cortical atrophy

2. Loss of sulci superiorly

3. Periventricular white matter changes from transependymal flow and a T2-signal hypointense flow void in the cerebral aqueduct

Front 65

Is OSA an independent risk factor for cognitive impairment?

Back 65

Yes; suggested pathomechanism have included hypoxemia resulting in subsequent reperfusion brain injury, endothelial dysfunction causing a vascular-type dementia, and excessive daytime somnolence affecting daily functioning

Front 66

What is Ganser syndrome?

Back 66

A form of dementia where the answers are always nearly correct but still incorrect, may occur in pseudodementia but is also present with malingering

Front 67

What is a fugue

Back 67

A psychiatric disorder characterized by reversible amnesia for personal identity that may last hours to months