Neurodegenerative disease Flashcards
How does the tremor of Parkinson’s change when a patient is asleep?
It disappears (paradoxical despite being a resting tremor). Most tremors caused by damage to the basal ganglia will disappear during sleep
What diseases cause choreiform movements?
Wilson and Huntington disease
Damage to what part of the brain leads to intention (or kinetic) tremors?
The cerebellum (can also result from damage to cerebrospinal tract)
What are risk factors for Alzheimer’s disease?
Age, female sex, history of severe head trauma, and Down syndrome.
ApoE4 (Chr 19) is a genetic risk factor.
Early-onset AD is associated with amyloid precursor protein (APP) (Chr 21), presenilin 1 (PS1) (Chr 14), and presenilin 2 (PS2) (Chr 1)
What area of the brain is primarily affected by progressive supranuclear palsy (PSP)?
Primarily the frontal lobe
What are the primary characteristics of progressive supranuclear palsy (PSP)?
Limitations in vertical gaze, extrapyramidal rigidity, gait ataxia, dementia, and frequent or early falls
What gene abnormality is found in Huntington disease?
More than 40 CAG repeats in the HD gene on chr 4
What genes have been implicated in Parkinson disease?
SNCA (alpha-synuclein), PARK2 (E3 ubiquitin ligase), UCHL1 (ubiquitin carboxyterminal esterase L1) PINK1 (PTEN-induced putative kinase 1), and LRRK2 (leucine-rich repeat kinase 2)
What PD patients are good candidates for deep brain stimulation?
Patients without significant active cognitive or psychiatric problems who have medically intractable motor fluctuations, intractable tremor, or intolerance of medication adverse effects
What are hereditary causes of chorea?
Huntington disease, neuroacanthocytosis, and Wilson disease
What is Marchiafava-Bignami syndrome?
Frontal-type dementia, seizures, and pyramidal signs; focal demyelination and necrosis of the corpus callosum. Due to alcohol consumption.
What are typical signs of cerebellar degeneration?
Gait ataxia greater than limb ataxia, dysarthria, typically no nystagmus. Due to alcohol consumption.
What is Lhermitte’s sign?
A tingling, electric shock sensation of the spine when the patient flexes the neck - seen in MS
What areas of the brain does MS most commonly affect?
Periventricular white matter, juxtacortical regions, corpus callosum, and the cerebellar peduncles
What change in visual evoked potentials can be used in suspected MS to document evidence of old optic neuritis?
Increased latency of the P100 wave on the affected side
What is the cause of neuromyelitis optica (Devic disease)?
Antibodies to the aquaporin-4 channel. Characterized by transverse myelitis and optic neuritis. Poor prognosis with most patients developing paralysis and blindness in the long term
What is posterior reversible encephalopathy syndrome (PRES)?
A leukoencephalopathy caused by rapidly developing hypertension and eclampsia or is due to immunosuppressants used in organ transplant (e.g. tacrolismus and cyclosporine). Characterized by acute confusional state and cortical visual loss (blindness with preserved pupillary reactivity).
What is Wallerian degeneration? vs neuronal (axonal) degeneration?
Wallerian: Disintegration of axons and myelin distal to the site of injury.
Neuronal: distal dying of the axons and loss of myelin after damage to the cell body of the neurons
What type of urinary incontinence is most commonly seen in MS?
Urge incontinence due to a spastic bladder
What are common symptoms of progressive supranuclear palsy?
Parkinsonism, supranuclear impairment of eye movements (vertical gaze typically affected more prominently than horizontal gaze), and impaired postural reflexes
Are the extrapyramidal features of idiopathic PD (e.g. resting tremor, rigidity) usually symmetric or asymmetric?
Asymmetric
What language deficits and common to Alzheimer disease?
Decreased fluency, dysnomia, and transcortical sensory aphasia (reduction in ability to understand complex linguistic structures). Repetition remains intact
What is general paresis?
It is a severe neuropsychiatric disorder caused by chronic meningoencephalitis that leads to cerebral atrophy in late-stage syphilis. Degenerative changes are associated primarily with the frontal and temporal lobar cortex. It is characterized by dementia, delusions, dysarthria, tremor, myoclonus, seizures, spasticity, and Argyll Robertson pupils
What are Lewy bodies?
Intracytoplasmic, eosinophilic inclusions with poorly staining halos surrounding them
How does speech change in Parkinson disease?
The clarity and volume decrease (develop hypophonia). Handwriting also becomes increasingly smaller and less legible (micrographia)