Acute ND and Neurodevelopmental Disorders

Patho exam 3 quiz 2

Neurodevelopmental disorders

Impairments of brain function occurring as brain develops

• language, coordination, attention, behavior

Brain tumors

Space-occupying lesions that cause increase in ICP

Benign and malignant tumors can be life-threatening

• unless accessible and removable

Gliomas form the largest category of primary malignant tumor

Primary malignant tumors

Rarely metastasize outside the CNS (BBB)

Secondary brain tumor

Metastasizing from breast or lung tumor

Brain tumors patho

Usually not well defined

Invasive and have irregular projections into adjacent tissue

• usually inflammation around the tumor

Brain tumor etiology

Brainstem and cerebellar tumors common in young children

Adults; more frequently in the cerebral hemispheres

Brain tumor signs and symptoms

Seizures often a sign

Headaches, vomiting, unilateral faial paralysis

Do not cause systemic signs of malignancy

Brain tumor treatment

Surgery if accessible 

Chemotherapy and radiation

Meningitis

Inflammation of the meninges

Different age groups are susceptible to different causative organisms

• may be secondary to other infections

Meningitis in young adults

Most often due to haemophiles influenza bacteria (more often in the fall or winter)

Meningitis in children and young adults

Neisseria meningitides or meningococci

• Classic meningitis pathogen

• Frequently carried in the nasopharynx of asymptomatic carriers

• Spread by respiratory droplets (late winter/early spring)

Meningitis in older adults

Strept pneumonia is the major cause

Meningitis signs and symptoms

Sudden onset

Back pain

Photophobia 

Nuchal rigidity

Kerning sign

Brudzinski sign

Meningitis diagnosis

Examination of CSF (lumbar puncture)

Identify causative organism

Meningitis treatment

Aggressive antimicrobial therapy

Specific treatment for ICP and seizures

Glucocorticoids (that stop inflammation)

Vaccines are available

Brain Abscess

Pus-filled swelling in the brain, caused by bacteria/fungi entering brain tissue after infection/injury

Localized infection

Necrosis of brain tissue and surrounding edema

Can spread

Treatment: Surgical drainage and antimicrobial therapy

Encephalitis

Infection of the parenchymal (nerve or glial cells) or connective tissue of the brain/spinal cord

• necrosis and inflammation development in brain tissue 

  • Viral, bacterial, autoimmune, or allergic rxns

Encephalitis early signs

Severe headache, stiff neck, lethargy, vomiting, seizures, fever

Encephalitis diagnosis

Imaging tests (CT, MRI) CSF analyses

Tetanus

Caused by Clostridium tetani

Spores can survive in soil (years)

Wound

Exotoxin enters nervous system

• jaw spasms and stiffness

• Difficult swallowing

Herpes Zosters (shingles)

Caused by varicella-zoster virus in adults

Years after infection of varicella

Affect a CN or dermatome

Pain, paresthesia, vesicular rash

Lesions and pain persist for a few weeks - could even be months/years

Concussion

Minimal brain trauma

Reversible interference with brain function

Results of mild blow to the head or whiplash type injury

Amnesia and headaches

recovery usually 24 hours without permanent damage

Contusion

Bruising of brain tissue, rupture of small blood vessel (inflammation and edema)

Blunt blow to head-possible residual damage

Closed head injury

Skull is not fractured in injury

Brain tissue is injured and blood vessel may be ruptured

Extensive damage may occur when head is rotated

Open head injury

Involved fractures or penetration of the brain

Depressed skull fracture

Involve displacement of a piece of bone below the levels of the skull

Compression of brain tissue

Blood supply to area often impaired - pressure to brain

Basilar fractures

Occur at the base of the skull

Leakage of CSF through ears or nose is possible

May occur when forehead hits the windshield

Contrecoup injury

Area of the brain contralateral to the site of the direct damage is injured

May be secondary to acceleration or deceleration injuries

Think rebound injury

Head injury

Trauma to brain tissue

Causes loss of function in part of body controlled by the area of the brain

Celld damage and bleeding lead to inflammation and vasospasm around injury site

Hematoma

Classified by their location in relation to the meninges

ADD IN DIF TYPES OF DURAL BLEEDS slide 26

Head injury signs

Focal signs (neurological symptoms that affect a specific area of the brain or nervous system)

General signs of increased ICP

Seizures

Cranial nerve impairment may occur

Otorrhea or rhinorrhea

Fever

Head injury treatment and diagnosis

MRI and CT

Glucocorticoids agents (decrease edema)

Antibiotics (reduce infections)

Surgery (to reduce ICP)

Oxygen (to protect remaining brain tissue)

Spinal cord injury

Damage can be temp or permanent

1. Laceration of nerve tissue by bone fragments or complete transection or crushing of cord

2. Partial transection or crushing of the cord or bruising

3. Prolonged ischemia and necrosis

Spinal cord injury treatment

PT/OT

Hydrocephalus

Excess CSF accumulates at skull

• more production than reabsorption

If cranial sutures have not closed, head enlarges (infant)

Damage depends on the rate of the pressure

Non-communicating/obstructive Hydrocephalus

Flow is blocked

Communicating Hydrocephalus

Problem on absorption on CSF through subarachnoid villi

Hydrocephalus signs

Increase in CSF

Scalp veins look dilated

Hydrocephalus diagnosis

Diagnostic test

CT ir MRI

Hydrocephalus treatment

Surgery or shunt

Dementia

Chronic (persistent) disorder of mental processes

Caused by brain disease or injury

Memory disorders, personality changes, impaired reasoning

Slow, progressive onset with impairments in abstract thinking and memory loss

Dementia causes

Illnesses

Head trauma

Alzheimer disease (AD)

• more than 50% of cases

Vascular dementia

• Changes in thinking following series of small strokes

Dementia clinical man

Slow decline of cognitive function

Increasing cognitive impairments over years

Progressive changes in mobility

Dementia diagnosis

Compete physical and neurologic exam

Dementia treatment

regular monitoring of health and cognitive status

Reinforcing orientation to person, place, time

Encouraging establishment of advance directives

CAN ONLY DELAY

Multiple Sclerosis

Progressive demyelination of the neurons in the brain spinal cord and cranial nerves

• autoimmune

Loss of myelin interferes with conduction of impulses in affected fibers

• may effect motor, sensory, and autonomic system

Multiple Sclerosis lesions

Inflammatory response

Loss of myelin in white matter of brain or spinal cord

Multiple Sclerosis plaques

Larger areas of inflammation and demyelination

Develop later, become visible in X-Ray

Multiple Sclerosis Recurrence

Initial inflammation may subside

Neural fct may return to normal for short period of time

Each recurrence causes additional areas of the CNS to become affected

Multiple Sclerosis treatment

No known treatment

Parkinson Disease

Idiopathic, chronic, progressive degenerative disorder of CNS

Has motor, nonmotor, neuropsychiatric manifestations

Affected indvs over 50 years

Parkinson Disease symptoms

Tremors

Bradykinesia

Rigidity

Postural instability

Parkinson Disease decreased risk

Cigarette smoking, caffeine intake

High blood urate levels

Parkinson Disease pathogenic mechanisms

Proteolytic stress

• accumulation and aggregation of proteins

• Lewy bodies

Oxidative stress

• Generation of ROS

• Depletion of glutathione, increase of iron

Mitochondrial dysfunction

• Decreased mitochondrial activity

Inflammation

• Overactivation of microglia

Parkinson Disease clinical man

Insidious onset

Motor features

• tremors

• Rigidity

• Cogwheeling

nonmotor

• Fatigue, pain, autonomic dysfunction

Parkinson Disease treatment

Pharmacologic replenishment with dopaminergic drugs

Deep brain stimulation

Supportive therapies

Huntington Disease

Progressive, incurable, neurodegenerative disease of brain, autosomal dominant inherited

Causes uncontrolled involuntary movements, dementia, and behavior changes

Onset of symptoms: 35-44 yrs

• duration: 19yrs avg

Primary cause of death:

• Pneumonia, CVD

Huntington Disease patho

Gross atrophy in caudate nucleus and putamen accompanied by selective neuronal loss and gliosis

Neuronal loss in cerebral cortex

Varying degrees of atrophy in other areas in midbrain and cerebellum

Shrinkage of brain (in volume)

Huntington Disease cause

Mutation in Huntington gene (HTT) on short arm of chromosome 4

Huntington Disease clinical man

Involuntary movements

• Parkinsonian features

• Akinetic-rigid syndrome

• Dysarthria

• Dysphagia

• tics

Cognitive impairments

Behavioral changes

Huntington Disease diagnosis

Family history

Clinical presentation

MRI or CT

Huntington Disease treatment

Reduce symptoms and improve quaility of life

Amyotrophic Lateral Sclerosis (ALS)

Progressive neurodegenerative disease

Causes weakness, disability, and death within 3-5 yrs

Knowns as Lou Gehrig’s disease

Neurodegeneration

• upper motor neurons in corticospinal tract

• Lower motor neurons in anterior horn cells of spinal cord

ALS risk factors

Age and family history

ALS cause and patho

No direct cause

Similar to prion disease or malignancy

Motor axons die by Wallerian degeneration

ALS pathways lead to cell death

Oxidative stress

Mitochondrial dysfunction

Defect of axonal transport

Abnormal growth factor

ALS clinical man

Insidious onset

Slowly progressive, painless weakness in one or more body parts

Upper motor neuron (UMN) signs and symptoms

Lower motor neuron (LMN) signs and symptoms

Bulbar dysfunction

ALS diagnosis

Based on clinical man

Tests

• electromyography

• MRI/CT

• Blood

Seizures

Abnormal electrical discharges within brain

Results in involuntary movement and/or behavior and sensory alterations

Seizure clinical man

Loss of conscious awareness of environment

Varying patterns of muscular rigidity and relaxation

Aura

Tonic Phase Seizure (15-60 seconds)

Muscular rigidity

Sudden loss of consciousness

Pupils fixed and dilated

Increased metabolic demands

Hypoxia

• Skin pallor and cyanosis

Urinary and bowel incontinence

Clonic phase seizures (60-90 sec)

Alternating muscular contraction and relaxation in extremities

Hyperventilation

Eyes roll back, froth at mouth

Postical period seizure

Decreased LOC; sleepy

Quiet and relaxed breathing

Gradual regaining of consciousness

Status epilepticus clinical man

Life-threatening condition

Enhanced and sustained electrical activity over 30 minutes

Increased neuronal excitation with reduced inhibition

Seizure Diagnosis

Laboratory tests

• Complete blood cell count

• Blood chemistry

• Urine culture

• Lumbar puncture

EEG

• Lead level, toxicology screening

• CT scan or MRI and angiography

Treatment

• Antiseizure medications

• Surgical intervention

Seizure treatment

Antiseizure meds

Surgical intervention