Neuro E2- Cognitive disorders

Delirium or Dementia?

• acute onset

• responds to some stimuli appropriately

• appear sleepy, disoriented, and inattentive

• level of consciousness may be impaired

Delirium

Delirium or Dementia?

• chronic onset, worsening over time

• usually appear normal but confused

• normal level of consciousness

• impairment in the content of consciousness

Dementia

What is the generalized loss of memory, psychogenic or organic in nature, with inability to learn new material?

Amnesia

What score on Folstein’s MMSE requires additional testing?

less than 24/30

What is disorder of articulation where basic language is intact (issue w/ speaking, not the content)?

Dysarthria

What is a true language disturbance that demonstrates impaired production and/or comprehension of spoken language?

Aphasia

What area of the brain is the center for language use, that processes input from both visual and auditory sources (reading/hearing)?

Wernicke’s area

What is the language pathway?

visual or auditory cortex (listening) → wernicke’s area (understanding) → Broca’s area (speaking ) → motor cortex (speaking)

What aphasia results form a lesion in the posterior temporal region (receptive aphasia)?

Wernicke’s aphasia

What aphasia results from a lesion in the frontal lobe (expressive aphasia)?

Broca’s aphasia

What type of aphasia?

• does not understand what is said or written

• unable to repeat what they hear

• speech is fluent (grammatical) but disordered

• can’t convert thoughts into meaningful language

• unaware that their responses are nonsense

• receptive

Wernicke’s

What type of aphasia?

• understands what they hear but produces few words with many pauses

• unable to repeat what is said to them

• intonation is flat and monotone

• repetition of words and phrases are impaired

• normal content with no grammar

• aware of deficit

• expressive

Broca’s

What type of aphasia?

• both receptive & expressive elements

• loss of ability to comprehend spoken/written language & express language

• may retain some comprehension & fluency

• usually correlates w/ infarction of MCA

• can be early manifestation of neoplastic, inflammatory, or degenerative disorder

Global

What kind of amnesia?

• can’t remember emotionally charged events

• can remember objective facts and events, but not the whole trauma

Psychogenic amnesia

What kind of amnesia?

• can’t remember objective facts

Organic amnesia

What kind of amnesia?

• loss of memory for events immediately prior to the disorder

• ex: occurs with head trauma

Retrograde amnesia

How would the acutely confused patient respond to the recall & memory portion of MMSE?

unable to learn new material due to impaired level of attention or consciousness

How would the demented patient respond to the recall & memory portion of MMSE?

problem with recent memory, remote memory is intact

What are red flags symptoms?

progressively declining neuro exam or LOC, pupillary asymmetry, seizures, repeated vomiting, double vision, worsening headache, disorientation, unusual behavior / confused or irritable, slurred speech, unsteady on feet, weakness/numbness in arms or legs

What are causes of delirium?

Dementia

Electrolyte disorders

Lung, liver, kidney, brain dysfunction

Infx

Rx drugs

Injury, pain, stress

Unfamiliar environment

Metabolic

How does alcohol intoxication present?

nystagmus, dysarthria, limb, gait ataxia (drunk = delirium)

Does alcohol level predict symptoms?

no

What symptoms are seen with alcohol withdrawal?

early (w/in 6 hrs): insomnia, tremulousness, mild anxiety, GI upset, HA, diaphoresis, palpitations

day 2: generalized tonic clonic seizures & hallucinations

day 2-4: delirium tremens (DTs) - hallucination, disorientation, tachycardia, HTN, hyperthermia, agitation, diaphoresis (lasts 1-5 days)

What is the treatment for alcohol withdrawal?

benzodiazepines- Diazepam

correct fluid, electrolyte, glucose, & thiamine abnormalities

What endocrine emergencies can cause delirium (altered level of consciousness)?

hypothyroidism (tx w/ levothyroxine)

hyperthyroidism (tx w/ antithyroid drugs, BBs)

hypoglycemia (tx w/ IV dextrose)

hyperglycemia (tx w/ insulin, IVF, and K if DKA)

What electrolyte abnormalities can cause delirium?

Hyponatremia: +HA, N, V, seen in athletes who replace fluids w/ only water

Hypocalcemia: +paresthesias, Trousseau & Chvostek signs

What is the treatment for hyponatremia?

water restriction & IV hypertonic saline; slowly raise level to 125-130

***avoid central pontine myelinolysis

What is the treatment for hypocalcemia?

IV calcium gluconate and seizure precautions

What environmental conditions can cause delirium?

heat exhaustion & heat stroke

What condition?

• thiamine deficiency in chronic alcoholic

• causes delirium, ophthalmoplegia/nystagmus, gait ataxia

• ataxia may not be fully reversible

• deficits in learning & memory can follow

Wernicke’s encephalopathy

What is the treatment for Wernicke’s encephalopathy?

Thiamine replacement (give BEFORE glucose bolus)

What should all patients with undiagnosed AMS, oculomotor disorders, or ataxia receive?

Parenteral thiamine

What nutritional disorder?

• due to poor intake, often w/ pernicious anemia

• presents with mild confusion to dementia to psychosis

Vit B12 deficiency

What condition?

• accumulation of ammonia in liver failure

• delirium, asterixis, seizures, coagulopathy

• requires lactulose & correct coagulopathy

Hepatic encephalopathy

What condition?

• renal failure

• causes delirium

• requires dialysis & renal transplant

Uremia (elevated BUN)

What psychiatric disorders can present with delirium?

psychoses, severe depression, acute anxiety

What condition?

• infection of meninges- invasion of bloodstream, crosses BBB

• direct contiguous spread- sinusitis, OM, lungs, UTI, wounds

• MC- s. pneumoniae

Meningitis

What is the classic triad of meningitis?

fever, nuchal rigidity (neck stiffness), mental status change

What is the workup for meningitis?

Kernig’s & Braduzinki’s tests

MRI- edema, inflammation

LP- cloudy CSF, high protein, high lactate, low glucose, many WBCs

What condition has the following CSF analysis?

• Opening pressure: 200-300

• WBC: 100-5000; >80% PMNs

• Glucose: < 40

• Protein: > 100

• Microbiology: specific pathogen demonstrated in 60% of gram stains & 80% of cultures

Bacterial Meningitis

What condition has the following CSF analysis?

• Opening pressure: 90-200

• WBC: 10-300; lymphocytes

• Glucose: normal or reduced in LCM & mumps

• Protein: normal or slightly elevated

• Microbiology: viral isolation, PCR assays

Viral meningitis

What condition has the following CSF analysis?

• Opening pressure: 80-200

• WBC: 0-5; lymphocytes

• Glucose: 50-75

• Protein: 15-40

• Microbiology: negative findings

Normal values

What is the MCC of meningitis?

viral

What type of meningitis?

• slower onset & less severe sx

• MC enterovirus, also HSV, VZV, mumps, HIV

• CSF: slightly elevated WBC w/ inc lymphocytes (not neutrophils); normal glucose; slightly elevated CSF protein

viral meningitis

What is the treatment for meningitis?

begin abx ASAP if bacterial suspected (regardless of what it is)

if viral suspected - acyclovir

considere steroids w/ first dose - decadron

airway support, O2, intubate, fluid hydration, Tylenol for fever

What should be done BEFORE starting treatment for meningitis?

blood cultures & LP

What condition?

• infection of brain tissue; MC viral

• AMS, personality changes, hemiparesis, focal seizures, autonomic dysfunction, ataxia, dysphagia

Encephalitis

What kind of encephalitis?

• fecal - oral transmission

• MC in summer & fall

• herpangina: hand, foot, mouth; mild sx (except for enterovirus 71- assoc w/ rhomboencephalitis)

• low mortality (except for enterovirus 71)

Enterovirus encephalitis

What kind of encephalitis?

• MCC of fatal encephalitis in US

• human-human transmission or reactivation of latent virus

• HSV, VZV

• start tx as early as possible - acyclovir

HSV encephalitis

What kind of encephalitis?

• transmitted by birds and mosquitoes

• late summer & early fall

• increased severity w/ advanced age

• supportive treatment

West Nile virus encephalitis

What kind of encephalitis?

• relatively common world wide

• transmission through infected animal bites

• presents w/ encephalitis or paralysis

• fatal unless vaccine & antiserum administered before sx occur

Rabies encephalitis

What is the workup for encephalitis?

Head CT +/- contrast; MRI

LP - search for elevated ICP, obstructive hydrocephalus, mass effect

EEG- diffuse slowing

CSF analysis- protein normal or slightly increased, lymphocytes present, glucose usually normal

What should be performed before LP in encephalitis evaluation bc of risk of brain herniation?

CT

What is the treatment for encephalitis?

airway management, fluid/elyte balance, nutrition, avoid & tx secondary infx, tx hyperpyrexia, manage ICP (head elevation, diuresis, mannitol, steroids, seizure precautions)

What condition?

• plasmodium species infx

• can present with delirium, seizures, focal neurological abnormalities

• organisms seen in thick & thin blood smear

• treatment based on geography,

• high mortality if seizures or coma

Cerebral malaria

How does CSF in cerebral malaria appear?

increased pressure, slightly elevated protein, monocytes

What condition?

• due to a sudden increase in BP

• if NO hx of HTN, sx appear at lower BP than a patient w/ HTN

• BP readings does not necessarily correlate w/ sx

Hypertensive encephalopathy

What is the treatment for hypertensive encephalopathy?

lower BP w/ labetalol or nitroprusside (avoid in stroke pts)

What condition?

• trauma induced alteration in mental status ± LOC

• severity of trauma associated with severity of symptoms

• hallmark symptoms: confusion & amnesia, can last minutes-hours

• early sx: HA, dizziness, lack of awareness of surroundings, N, V

• later sx: mood/cognitive disturbances, sensitivity to light and noise, sleep disturbances

Concussion

What increases morbidity in post-concussion syndrome?

repeat concussions

What are risk factors for delirium?

advanced age, preexisting cognitive deficit, prior episode of delirium, polypharmacy, alc/drug dependence, psychoactive drugs (benzos, anticholinergics, narcotics), vision/hearing deficits, preoperative complications, immobility, severe comorbidity

What should be given for hyperactivity in acutely confused patients?

Haloperidol (Haldol)

What condition?

• progressive decline in cognitive function beyond what might be expected from normal aging

• normal level of consciousness

• recent memory usually markedly affected

• sx involve diminution of cognitive skills & learned tasks and slowly progress to include personality changes & intellect

Dementia

What is the MC form of dementia?

Alzheimer’s disease

What is the workup for dementia?

non contrast CT or MRI, screen for depression, check vit B12 levels, thyroid function tests, syphillis testing if suspicion high, genetic markers to confirm if strong fhx

(apolipoprotein E epsilon 4 allele not currently recommended)

The following DSM V criteria is for what condition?

• cognitive impairment in at least 1 of the following: learning/memory, language, executive function, complex attention, perceptual motor function, social cognition

• must be acquire, represent significant decline, & interfere w/ independence

• does not occur exclusively during course of delirium (always present)

• not better accounted for by another disorder

Dementia (Major neurocognitive disorder)

Who is Alzheimer’s disease MC in?

F > M; prevalence 50% by age 85

What condition?

• progressive degenerative disorder (over 5-20 years)

• major sx: gradual development of forgetfulness

• progresses to disorientation to time, then place

• mobility, behavior, temperament, & conduct decline

• restless & agitated → depressed

Alzheimer's disease (AD)

What is the only way to definitively diagnose AD?

postmortem

What 3 microscopic changes are seen with AD?

neurofibrillary tangles (tau), cortical atrophy (pyramidal layer of hippocampus), & amyloid plaques

How does the brain appear in advanced stages of AD?

diffusely atrophied & weight reduced by ≥20%

ventricles enlarged, cerebral atrophy w/ cerebral sulci widened

How do AD patients appear early on in the disease?

non focal neuro exam: short term memory abnormal, speech hesitant, apraxia/visuospatial disorientation (gets lost easily)

& short, slow. shuffling steps, flexed posture, wide base, & difficulty initiating walking

How do AD patients appear later in the disease?

language & cognition decline; paranoia, hallucinations, delusions, pt becomes mute, bedbound, & incontinent; seizures, extrapyramidal rigidity/bradykinesia,

primitive reflexes emerge (sucking & grasping)

What workup can be done for AD?

MRI, CT, EEG (diffuse slowing), PET scan (diminished activity in temporal lobe), neuropsych testing (dec executive function)

correct dx made 90% of time w/ clinical data, time course, & CT/MRI

What are treatment options for AD mild-mod sx?

acetylcholinesterase inhibitors: donepezil, rivastigmine, galantamine

antipsychotics, antidepressants, anxiolytics

What are treatment options for AD severe sx?

memantine (Namenda)- glutamate receptor antagonist

Can treatment reverse existing deficits or arrest progression in AD?

no

What are SEs of acetylcholinesterase inhibitors?

N/V/D, dizziness, elevated LFTS (*get LFT before starting)

What are alternative therapies for AD?

Vit E, selegiline, ginkgo biloba

What occurs 5-20 years after AD onset?

death- infection & inanition (loss of social, moral, or intellectual vitality/vigor)

What are Lew bodies?

intracytoplasmic inclusions in cortex & brainstem associated with dementia (no tangles or plaques)

What condition?

• progressive dementia- alzheimers & late parkinsonian features

  • TRAP, amnesia, visuospatial disorientation, aphasia, fluctuations in alertness, etc

• diffuse involvement of cortical neurons w/ lewy body inclusions & absence of inconspicuous number of tangles/plaques

• psychotic features- hallucinations, delusions

Dementia w/ lewy bodies

What is the 2nd MCC of dementia?

dementia w/ lewy bodies

What should be avoided in dementia with Lewy bodies?

antipsychotics (more sensitive)

Psychotic features such as confusion, hallucinations, & paranoid delusions are seen in which condition?

dementia w/ Lewy bodies

What is the treatment for Dementia w/ Lewy bodies?

L-dopa for PD features for a limited time (can cause delirium/hallucinations or not have a consistent response)

What is the 3rd MCC of dementia?

vascular (multi-infarct) dementia

What condition?

• multiple infarcts in subcortical white matter, basal ganglia, or thalamus

• usually have hx of HTN or CV risk factors

• step wise progression; new infarct = new deficit

  • memory loss, slow thinking, balance issues, dysarthria, emotional lability, absent mindedness, etc

Vascular / Multi-Infarct Dementia

How was vascular/multi infarct dementia appear on MRI?

small subcortical lucencies / lacunar infarcts

What is the treatment for vascular dementia?

Donepizil, Memantine

manage HTN/CV risk factors- CCBs (nimodipine), anti-platelet drugs, regular exercise & healthy diet

What condition is most commonly mistaken for dementia?

depression

Dementia or depression?

• unaware of extent of deficits

• no complaints of memory loss (don’t know they forgot)

• few vegetative sx (up & moving when they are able)

• worse at night

• abnormal neuro exam & labs

Dementia

Dementia or depression?

• aware of deficits

• complains of memory loss (knows they forgot)

• prominent vegetative sx

• not worse at night

• normal neuro exam & labs

Depression