17 Dementia Flashcards
(23 cards)
Frontotemporal dementia
40-60y, Tau related, Altered eating/sexual/speech/disinhibition/primary progressive aphasia/personality/language/memory impairment, normal visual spatial skills, associated with motor neurone disease, sweet tooth, sometimes autosomal dominant, Treat SSRI (paroxetine), trazodone?,
Normal arithmetic (calculation) ability
*Pick’s disease: A frontotemporal subtype with language difficulty, Pick bodies -> Tau
*Semantic dementia: inability to name objects, recognize faces (prosopagnosia) using higher subtitudes ( apple -> fruit) left perisylvian region/parietal (anterior temporal atrophy)
*If there are UMN+LMN like fasciculations or rapid reflex, it is called FTD-MND and should do EMG.very poor prognosis.
Alzheimer
Most important risk factor: FH + others: APOE4, Dawn, trauma, vasculopathy
Memory/aphasia/apraxia/agnosia/executive function, difficult word finding, poor spelling, spatial awareness
Neurofibrillary tangles, B amyloid
Treat: acetylcholinesterase inhibitors: Donepezil, rivastigmine, galantamine -> memantine
Large ventricles+sulci+ shrunk gyri
Lewy body
ognitive/fluctuating attention/visual/ auditory hallucination/delusion/sleepy /parkinsonism/ falls
Lewy body ( Alpha synuclein )
Treat: Donepezil -> rivastigmine -> galantamine
Avoid anti psychotics.
Normal Hydrocephalus
Dementia, Ataxia, incontinence
Magnetic gait, planning and organization of complex tasks disturbance
Disturbance of fine motor control, sometimes parkinsonism
Can happen post meningitis or SAH
Hydrocephalus + ventriculomegaly + NO sulcus enlargement
Raised CSF resistance on CSF infusion
Diagnosis: LP (after CT)
Treatment: Definitive shunt, others: repeated LP, acetazolamide
First remove 30cc of CSF as test to see if gait improves.
Parkinsonism
Loss of dopaminergic transmission,
Earliest sign: REM behaviour disorder
Asymmetric tremor, rigidity lead pipe cogwheel, bradykinesia, turning en bloc, difficulty initiating movement, shuffling steps with reduced arm swinging and stride, tremor more marked at rest 3-5HZ, pill rolling in thumb and index, hypophonic voice
Micrographia, flexed posture, drooling, psychiatric, olfactory impair
*idiopathic is asymmetrical, while other causes are usually bilateral
Basal ganglia Lewy body ( Alpha synuclein )
Diagnosis: Consider SPECT
L-dopa Can go up to 4 doses a day
Off periods: apomorphine
Supplement to L dopa: Amantadin, entacapone, Ropinirole (gambling, pramipexole ) , Rasagiline?
Parkinsonism drug that doesn’t cause gambling: Procyclidine
Parkinson, Hallucination, Paranoid: Clozapine ( D2 agonist , check cbc for neutropenia, constipation) , Quetiapine
Parkinsonism management
Non pharmacological: Physiotherapy, Occupational, Speech, exercise, psychological support
Motor affecting quality of life: Levodopa (Nausea, Orthostatic hypotension, more motor complications)
Motor Not affecting: Dopamine agonist (pramipexole, ropinirole (gambling), rotigotine, more hallucination, impulse control, sleepiness), Levodopa, Mao B inhibitor (selegiline, Rasagiline, Serotonin syndrome), COMT inhibitor (entacapone, tolcapone, prolonge L-dopa effect)
Parkinsonism drug that doesn’t cause gambling: Procyclidine
If patient becomes sick, continue medication to prevent neuroleptic malignant syndrome. if cannot take levodopa orally, can give rotigotine(dopamine agonist) patch.
Dementia: Rivastigmine
Orthostatic hypotension: Midodrine
Drooling: Glycopyrronium bromide, procyclidine
Sleepiness: Modafinil
Insomnia: Melatonin, clonazepam
Tremor: Procyclidine, orphenadrine, trihexyphenidyl, benztropine
If acute psychosis: Reduce ropinirole
Psychosis: Clozapine( D2 agonist , check cbc for neutropenia, constipation), Quetiapine
Dysphagia: Speech therapy, PEG feeding
Advance disease: Deep brain Stimulation
Levodopa/Carbidopa/Benserazide ->
1-Wearing off phenomenon: Dose fractionation, add dopamine agonist or MAOB
2-On-Off fluctuations: Sudden unpredictable changes between mobility and immobility, happens with advance disease, adjust meds and Deep brain stimulation
3-dyskinesia (chorea, Dystonic) happens at peak dose ->Reduce levodopa + add amantadine, Add dopamine agonist (stronger, gambling, Bromocriptine, Cabergoline (pulmonary/cardiac fibrosis), Ropinirole, pramipexole, apomorphine (injectable, Rescue therapy in off episode)
Proper for young patients worried about dyskinesia and lung fibrosis: Ropinirole
4-Freezing of gait -> Advanced disease, Cueing techniques, physio,DBS
5-apomorphine (injectable, Rescue therapy in off episode in advance disease
Drug induced parkinsonism causes and treatment
Causes:Anti psychotic (Haloperidol, Risperidone, Olanzapine), Anti emetic (Metoclopramide, Prochlorperazine) CCB, CO poisoning, MPTP: Treatment: Procyclidine, benztropine, tri hexyl phenidyll)
Vascular parkinsonism
Worse in legs
Corticobasal degeneration
Parkinsonism (asymmetric rigidity, bradykinesia) myoclonus, dystonia, alien limb phenomena, apraxia, dementia, speech
MRI: asymmetric cortical atrophy
Definitive diagnosis: Post mortem showing Tau proteins
DD: Parkinsonism, supranuclear palsy, Alzheimer atypical, stroke
Treatment: No cure, symptomatic management
Multiple system atrophy
symmetric Parkinsonism, cerebellar ataxia, autonom (hypotension orthostatic, erectile dysfunction, incontinence,) sometimes UMN, alpha synuclein, no response to levodopa (This has early ataxia/urinary incontinence and little tremor unlike parkinson), shy drager, no cure, supportive therapy
Progressive supranuclear palsy
dementia, parkinsonism, fall, supranuclear gaze palsy, impaired down gaze, diplopia on lateral gaze, dysarthria, difficult swallowing, BP postural drop, poor response to Levodopa
Midbrain atrophy + Large 3rd ventricle
Cruetz jakob disease
Dementia + Myoclonic + ataxia visual/pyramidal/extrapyramidal/akinetic mutism, startle response,
Prion (beta sheets) in basal ganglia, Diagnosis MRI Flair DWI more sensitive/specific than RTQuIC, 14-3-3 in CSF: High T2 signal posterior thalamus, putamen, caudate, cortical ribboning ( sulci), EEG ( do not biopsy even though it’s gold standard )
Sporadic: 85%, Old, 10% familial, dementia, EEG abnormal periodic spike, High amplitude sharp
New variant: young, myoclonus, depression, leg pain, primary reflexes, dysarthria, ataxia EEG normal, High signal thalamus(pulvinar), survival 1 year
Iatrogenic: Limb ataxia, short term memory, hallucination, receiving GH in 1980
Vascular dementia
Cerebral autosomal dominant arteriopathy with subcortical infarcts leukoencephalopathy
Multi infarct dementia
Step-wise process
CADASIL
migraine, FH, strokes at a young age, temporal, NOTCH3 chromosome 19 and skin biopsy
*careful, MELAS has very similar presentation (early cva, migraine, vomiting, short stature, seizure ) but has lactic acidosis and red ragged fibers.
MELAS
mitochondrial, encephalomyopathy, lactic acidosis, strokes
Other mitochondrial signs: diabetes, deafness, seizure, young stroke, retinal dystrophy, proximal myopathy, cardiomyopathy, arrythmia, FSGS ESRD, short stature, BMI
Mitochondrial
Heteroplasmy ( poor correlation genotype/phenotype)
MELAS, MERRF, Kearns sayre, Leber optic atrophy
MERRF
myoclonus epilepsy wpw vision hearing Diagnosis biopsy muscle ragged red fibers
Leber
30 man, central scotoma -> colour vision loss -> visual impairment within 1year, FH + , optic atrophy, absent macular reflex, narrow vessels
DIDMOAD
DI, DM, deafness, vision loss in childhood or adolscences
Kearns sayre
ptosis, Ophtalmoplegia, pigmentary retinopathy, cardiac conduction
Autosomal dominant optic atrophy
Childhood or early adolescence
Essential tremor
Autosomal dominant, FH+, postural tremor(when arms are outstretched or tried to do tasks tremor worsens), rhythmic, oscillatory, can be asymmetrical, head/face tremor , no parkinsonism signs such as finger pinching abnormality, low amplitude (4-6), improved with alcohol and rest, most common cause of titubation( head tremor) propranolol, primidone , deep brain stimulation
Test to differentiate from parkinson: 123I-FP-CIT-SPECT or datSCAN
