Neuro - Dementia & Neuromuscular Disorders

Front 1

What is dementia?

Back 1

A chronic and progressive decline in the cognitive ability of a patient that leads to a decline in their activities of daily living. Only if the cognitive problems have a direct impact on the patient's daily life can the diagnosis of dementia be made.

Front 2

What is the percentage of individuals with dementia between 65 & 70 years old? > 85?

Back 2

65-70 is 5%

> 85 is 45%

Front 3

Alzheimer's disease accounts for what percentage of dementia?

Back 3

50-70%

Front 4

What is delirium?

Back 4

Acute change in a patient's mental state usually secondary to some sort of metabolic condition. Symptoms include poor attention, disturbances in the sleep wake cycle, hallucinations, and waxing and waning of symptoms.

Front 5

Pathogenesis of Alzheimer's disease

Back 5

Initially, pathology is limited to the hippocampus and enterhinal cortex, with isolated memory dysfunction. It spreads to the temporal and parietal lobe, causing trouble remembering the names of things and frequently getting lost. When it spreads to the frontal lobes, there are difficulties with judgment. The average time of death from diagnosis is 7 years.

Front 6

What does the histology of Alzheimer's disease show?

Back 6

Considerable cortical atrophy, particularly in the limbic system. Loss of pyramidal cells, extracellular amyloid plaques (amyloid B protein), and intracellular neurofibrillar tangles.

Front 7

What mutations are present in familial forms of Alzheimer's?

Back 7

Mutations in the BAPP gene, pre-senilin 1, or pre-senilin 2.

Front 8

What accounts for the majority of the sporadic, late onset AD?

Back 8

Apolipoportein E4

Front 9

What is the earliest gross structural change seen in AD?

Back 9

Mesial temporal lobe atrophy

Front 10

After a stroke, what percentage of patients are demented?

Back 10

20-25%

Front 11

What is the structural changes seen in vascular dementia?

Back 11

Chronic hypoperfusion of white matter, multiple lacunar infarctions, large artery disease, and acute hemodynamic compromise.

Front 12

Dementia with Lewy Bodies

Back 12

Similar to Parkinson's disease. Progressive cognitive decline with a milder movement disorder. Patients have fluctuations of cognition, and recurrent complex visual hallucinations. They may develop REM sleep behavior disorder years before the onset of dementia.

Front 13

Where are Lewy bodies found in dementia with Lewy bodies? Where are they found in Parkinson's?

Back 13

In Parkinson's, they are found in the substantia nigra of the midbrain. In DLB, they are found in the cortex.

Front 14

What is the treatment for dementia with Lewy bodies?

Back 14

Dopamine treatment may improve Parkinson's symptoms, but worsen hallucinations. Neuroleptics may help hallucinations, but worse the Parkinson's symptoms. ACh inhibitors have been shown to be of benefit.

Front 15

Frontotemporal degeneration (Pick's disease)

Back 15

Neurodegeneration primarily of the frontal lobes. Patients often experience emergence of artistic talent. "Knife edge" gyral atrophy in frontal/temporal lobes. Pick bodies (tau +, straight filaments) can be seen with silver staining. Pick cells are swollen, achromatic neurons.

ACh esterase inhibitors are contraindicated; no treatment alters the course of the disease.

Front 16

Frontotemporal dementia

Back 16

A type of Frontotemporal degeneration with personality changes, impaired social conduct, emotional blunting, and loss of insight. Also neglect of personal hygene, varacious appetite, and hypersexuality.

Front 17

Primary progressive aphasia

Back 17

A type of frontotemporal degeneration affecting primarly the left frontal lobe. Effortful non-fluent agramatic speech with preserved understanding of language.

Front 18

Semantic dementia

Back 18

A type of frontotemporal degeneration primarily affecting the temporal lobe. Difficulty understanding individual word meanings. Speech is empty of content.

Front 19

Creutzfeld-Jacob Disease (CJD)

Back 19

Incidence of 1 in 1,000,000. Rapidly progressive dementia with startle myoclonus. Duration of illness before death is usually 4 months after symptoms develop. 85% is sporadic.

Front 20

New variant CJD

Back 20

Neuropsychiatric symptoms & ataxia before dementia; median age of death is 28 (CJD is 68); Duration of illness from development of symptoms is 14 months.

Front 21

Gerstmann Straussler Scheinler disease

Back 21

Prominent ataxia with later onset of dementia. Duration of illness from development of symptoms is approximately 5 years. Onset is usually 3rd or 4th decade with autosomal inheritance.

Front 22

Familial fatal insomnia

Back 22

Progressive insomnia, neuropsychiatric symptoms, dysautonomia, weight loss, then total insomnia and dementia. Duration is 18 months.

Front 23

Kuru

Back 23

Mainly affects the cerebellum and manifests as unsteady gait, tremors, and slurred speech. Dementia is either minimal or absent. Mood changes often present and patients would often laugh uncontrollably.

Front 24

Normal pressure hydrocephalus

Back 24

Impaired CSF reabsorption at the arachnoid villi. Symptoms include gait disturbance, dementia, and urinary incontinence. "Magnetic" gait is the first symptom. Dementia is due to subcortical cognitive defects including forgetfulness, decreased attention, inertia, and bradyphrenia. Imaging shows ventricular enlargement out of proportion to sulci atrophy, thinning of corpus callosum, and rounding of the frontal lobes. Shunt is treatment

Front 25

Amyotrophic lateral sclerosis (ALS)

Back 25

Purely a motor disorder. Affects peripheral and central motor neurons. Lower motor neuron signs include twitching, weakness, limb and tounge atrophy, dysplagia, and dysarthria. Upper motor neuron signs include hyperreflexia, increased gag reflex, and Babinki's sign.

Front 26

ALS treatment

Back 26

Riluzole and non-invasive ventilation increases survival by 6 months.

Front 27

Spinal muscular atrophy

Back 27

Autosomal recessive disease of lower motor neurons. Weakness is present, but no intellect or sensory loss. It is purely a motor disorder.

Front 28

Kennedy disease

Back 28

Spinobulbar muscular atrophy. X linked recessive with CAG repeat causing a defect in androgen receptor. Symptoms include muscle cramps, fasciculations, limb weakness, dysphogia, dysarthria, and some develop gynecomastia.

Front 29

What is the most common cause of peripheral neuropathy?

Back 29

Diabetes

Front 30

Peripheral neuropathy shows what distribution in the limbs? chest? face?

Back 30

Limbs - stocking and glove

Chest - shield

Face - mask

Front 31

Charcot Marie Tooth disease

Back 31

Greater distal than proximal weakness, atrophy, and numbness. It is the most common inherited neurologic disorder. Most commonly caused by disturbances of the peripheral myelin leading to uniform demyelination and homogenously reduced conduction velocities on NCS. Children will present with high foot arches and hammer toes.

Front 32

Peroneal neuropathy

Back 32

When at the fibular head may cause weakness of the foot dorsiflexion and numbness of the dorsum of the foot, and it has been associated with crossing the legs by compressing the peroneal nerve as it crosses the fibular head against the contralateral knee under the crossed leg.

Front 33

What are the possible causes of multiple mononeuropathies?

Back 33

Lupus and polyarteritis nodosa

Front 34

Guillain-Barre syndrome

Back 34

Acquired autoimmune syndrome characterized by ascending weakness of the lower and upper limbs, arreflexia, and elevated CSF protein with normal cell count. Frequently there is subjective sensation of sensory disturbance without evidence of sensory involvement on physical or electrodiagnostic examination. It may be preceded by infection, trauma, or vaccination. Patients usually recover fully.

Front 35

Chronic inflammatory demyelinating polyradiculoneuropathy

Back 35

2 or more months with proximal and distal motor weakness, elevated CSF protein, and acquired demyelinating features on EMG, such as conduction block and temporal dispersion. Treatment includes steroids, IVIg, and plasma exchange.

Front 36

Parsonage-Turner syndrome (brachial plexus neuritis)

Back 36

Acute onset of arm pain associated with patchy weakness and numbness. Often associated with diabetes mellitus, lupus, and vasculitis. At times there is a history of viral infection or vaccination. Some have noted anterior interosseous nerve involvement and patients cannot form a circle with forced closing of the tips of the thumb and index finger.