Neurological Disorders

Dementia

A group of symptoms caused by brain damage that is progressive

Causes a loss of cognitive functioning - thinking, remembering, reasoning

Occurs in older people, but not a normal process of aging

Dementia Mechanism

Neuron degeneration

Atherosclerosis (vascular)

Brain tissue compression

Brain Trauma

Genetic predisposition

Alzheimer's Disease

Progressive and irreversible neurological disorder affecting memory, thinking, and ability to do tasks, MOST COMMON type of dementia

Late Onset Alzheimer's

Accounts for 95% of cases and begins after 65 years, it is usually sporadic and is caused by an alteration in apolipoprotein E gene located on chromosome 19

Early Onset Familial Alzheimer's

Accounts for 5% of cases, caused by chromosome 21 (note: same chromosome that affects people with Down Syndrome, people with Down Syndrome are almost certain to develop Alzheimer's by middle age).

Alzheimer's Pathophysiology (Extracellular)

Deposition of beta-amyloid (neuritic plaques)

Alzheimer's Pathophysiology (Intracellular)

Accumulation of tau proteins (neurofibrillary tangles)

APP

Amyloid Precursor Protein, abnormal processing of this leads to Alzheimer's

Alzheimer's Signs

Behavioral changes - irritable, mood swings, hostility

Forgetfulness leading as progressive memory loss

Poor judgement

Alzheimer's Diagnosis

Only confirmed by brain biopsy, exclusion of other neuro disorders

Alzheimer's Treatment

No cure, anticholinesterase drugs temporarily improve condition

Team specialist approaches to support caregivers (occupational, speech, psychologists)

Parkinson's Disease

Progressive, neurodegenerative disease of melanin-containing dopaminergic neurons in substantia nigra, pars compacta (subdivision of substantia nigra)

SEVERE degeneration of basal ganglia (neurons that control voluntary musle movements and inhibit unwanted ones)

Idiopathic / Primary Parkinson's

Refers to the majority of Parkinson's cases, no known cause

Secondary Parkinson's

Environmental causes (pesticides), anti-psychotics,

Pseudoparkinsonism

Condition that mimics the effects of Parkinson's, but is not caused by the same neurodegeneration

Parkinson's Treatment

Dopamine agonists like Levodopa, MAO-B inhibitor, anticholinergic, team support

Huntington's Disease

Progressively debilitating neurodegenerative inherited disease (autosomal dominant disorder) on chromosome 4 or Huntington (HTT) gene

Doesn't manifest until older ~40s, progressive atrophy of the brain

Huntington's Disease Pathophysiology

Hyperkinetic disease that involves basal ganglia and frontal cortex

Depletion of GABA in basal nuclei (caudate nucleus and putamen)

Levels of acetylcholine in brain appear to be reduced (muscle contractions)

Huntington's Disease Signs

Mood swings, restlessness with choreiform (purposeless) movements in arms and face

Huntington's Disease Diagnosis

DNA Analysis

Huntington's Disease Treatment

No specific treatment, symptomatic therapy only

Huntington's Disease Mortality

Infections, heart disease, suicide

Amyotrophic Lateral Sclerosis (ALS) / Lou Gehrig's Disease

Rapidly progressive and fatal neurodegenerative disease of the upper and lower motor neurons with SOD1 gene mutation

ALS Pathophysiology

Not completely clear, but damage to glutamate uptake channels in astrocytes, Gulf War veterans higher risk (2x as likely), repeated trauma in athletes

No inflammation around nerves

ALS Upper Neuron Loss

Spastic paralysis and hyperreflexia

ALS Lower Neuron Damage

Flaccid paralysis and hyporeflexia

ALS Common Symptoms

Progressive weakness, loss of fine motor skills (stumbling and falling), death eventually by respiratory failure

ALS Treatment

No specific treatment, stem cell therapy is being researched

Riluzole slows further damage and edavarone can neutralize free radicals that cause nerve damage

Moderate exercise

Team approach

Cerebral Ischemia

Damage and manifestations depend on cerebral artery involved, global or local

Cerebral Hemorrhage

Increased ICP will cause local ischemia and generalized symptoms

Arteriovenous Malformation

Cause of hemorrhagic strokes, abnormal connection between veins and arteries

Transient Ischemic Attack (TIA)

Minor stroke, neurological function is regained quickly

Lass <1 hour from ischemic event

Caused by atherosclerosis (most common), partial occlusion of an artery, spasms, lesions

Visual disturbances, numbness, parasthesia, transient aphasia or confusion

Diagnosed by MRI, CT, angiography

Initiate stroke prevention therapy

Cerebral Vascular Accident (CVA)

Stroke, 5 minutes of ischemia with irreversible neuronal damage

Leading cause of disability and 5th leading cause of death in US, associated with hypoperfusion

Higher risk in African-Americans

Thrombotic Stroke

Arterial occlusions caused by thrombi

Embolic Stroke

Fragments of a broken thrombus

Hemorrhagic Stroke

Blood vessel rupture in the brain, caused by hypertension, aneurysms, arteriovenous malformation

3rd most common cause, high ICP

Contralateral motor and sensory deficit, initially flacid paralysis and then spastic paralysis develops weeks later

Hemorrhagic Stroke Symptoms

Abrupt hemiparesis (partial one sided weakness), visual loss, nystagmus, dysarthria, facial droop, ataxia, vertigo, aphasia, headaches, sudden decrease in consciousness

Hemorrhagic Stroke Treatments

Cardiovascular disease, platelet antiaggregant, statins, antihypertensive treatment, diabetic treatment, STOP smoking, weight loss

Bacterial Meningitis

Neisseria meningiditis or streptococcus pneumonia

Hematogenous (blood spread) or direct contagion

Bacterial Meningitis in Babies

Fever, sleepy and less responsive, unusual crying, stiff neck, dislike bright lights, vomiting, refusal of feeding, red or purple spots on skin

Bacterial Meningitis in Adults

Very similar to babies

Kernig Sign

inability to straighten the leg when the hip is flexed to 90 degrees, meningitis sign

Brudzinski Sign

Involuntary lifting of legs when neck is flexed, sign of meningitis

Meningitis Treatment

Rapid diagnosis and treatment to prevent death, aggressive antimicrobials, glucocorticoids to reduce cerebral inflammation, vaccines are preventative

Multiple Sclerosis (MS)

Autoimmune mediated disease that is progressive, inflammatory, and dymyelinates CNS

1 in 100,000, more common in women occuring at 20-40 years

Repeated remissions causes eventual neural degeneration with loss of function

Multiple Sclerosis Signs

Clumsiness and muscle weakness, paresthesia, optic neuritis (optic nerve inflammation with blurred or double vision), lhermitte sign, impaired gait, incontinence, dysarthria (weakness in speech muscles)

MS Diagnosis

Complete neuro exam, MRI for diagnosis and monitoring, CSF analysis

MS Treatment

None approved, research trials in progress, physical therapy with focus on individual manifestations