A Potpourri of Neurological Disorders

A Potpourri of Neurological Disorders
By:Stephanie Rodriguez MS RN

Chronic Neurological Problems
Headaches
* Classified based on characteristics of the headache
* Functional vs. Organic type
* May have more than one type of headache
* History & neurologic exam diagnostic keys

Not always chronic…be careful
Pattern
Tension
Migraine
Cluster
Site
Bilateral, basilar, band-like
Unilateral, anterior
Unilateral, occular
Quality
Squeezing, constant
Throbbing
Severe
Pattern
Cycles, years
Periodic, years
Remitting, relapsing
Duration
Days, weeks, months
Hours, days
30-90 min
Onset
Anytime
Prodrome, starts in AM
Nocturnal
Assoc. Sx
Stiff neck
N&V, photo/phono-phobia
Horner syndrome
ONSET: Not reliable or diagnostic

Horner’s Syndrome
HA: Essential History
* Onset this particular headache
* Character of pain, severity and duration
* Associated symptoms
* Prior history, pattern
* Original onset: prior testing, treatment
* Exertional aspects
* Other therapeutic regimens

Physical Exam
* Neurologic examination
* Inspect for local infections, nuchal rigidity
* Palpation for tenderness, bony swellings
* Auscultation for bruits over major arteries

Organic vs. Traumatic vs. Functional: Diagnostics
* CBC: underlying illness, anemia
* Chem panel: if associated vomiting, dehydrated
* U/A
* CT scan: for focal neurological signs, sinuses
* SED rate: if recent onset, > 50 yrs (temporal arteritis)
* No LP for suspected ICP; ↑ association with brain herniation (question from last time: ARTICLE

Don’t Miss It
* Caused by subarachnoid hemorrhage from an aneurysm or head injury
* “Worse headache of my life”
* Changes in LOC, focal neurological signs
* Highly correlated with CVA
* Untreated, 50 % mortality

Headache Teaching Guide
* Keep a calendar/diary
* Avoid triggers
* Medications (purpose, side effects)
o Imitrex: take med at 1st sign of HA, ↓blood flow to brain
* Stress reduction
o Dark quiet room, exercise, relaxation
* Regular exercise
* When to contact PCP

Multiple Sclerosis
Definition
MS is a chronic, progressive, degenerative disorder of the CNS characterized by disseminated demyelination of the nerve fibers of the brain and spinal cord.

Multiple Sclerosis
Etiology
* Cause is unknown.
o Possibly related to viral, immunologic, and genetic factors.
* Susceptibility appears to be inherited with 1st, 2nd, and 3rd degree relatives at slight increased risk.

Pathophysiology
* MS is characterized by chronic inflammation, demyelination, and gliosis in the CNS.
* Autoimmune process is orchestrated by autoreactive T cells (lymphocytes)
* Activated T cells migrate to CNS causing blood-brain barrier disruption.
* Subsequent antigen-antibody reaction within the CNS activates the inflammatory response leading to demyelination of axons, with plaque formation.

A: Normal Nerve Cell
B: Normal Axon
C: Myelin Breakdown
D:Myelin completely disrupted

U-tube: animation
* http://www.youtube.com/watch?v=eE_Y3KMYC0g

Disease Process: Early
* Myelin sheaths of neurons in the brain and spinal cord are initially attacked.
* Myelin sheath is damaged but nerve fiber still intact.
* Nerve impulses are still transmitted but patient may complain of weakness.
* Remission occurs if inflammatory process stops and myelin regenerates.

Disease Process: Late
* If remission does not occur, myelin disruption continues and axon is affected.
* Myelin is replaced by glial scar tissue which forms plaques in the CNS.
* Lacking myelin, nerve impulses slow, axons are destroyed, resulting in permanent loss of function.`

Symptoms
* Initially insidious with symptoms evolving over months to years.
* Systems affected depend on distribution of lesions.
* Disease process may be chronic and progressive or have periods of remission and exacerbation.
* Even in relapsing disease, overall trend is progressive deterioration of neurologic function.

Symptoms: Motor
* Weakness
* Paralysis of limbs, trunk or head.
* Diplopia
* Scanning speech
* Muscle spasticity

Symptoms: Sensory
* Numbness, tingling, parathesia.
* Scotomas (patchy blindness)
* Blurred vision
* Vertigo
* Tinnitus, decreased hearing
* Neuropathic pain
* Radicular pain

Symptoms: Cerebellar
* Nystagmus
* Ataxia
* Dysarthia
* Dysphagia

Symptoms: Emotional
* Emotional stability may be affected
* Depression common co morbidity
* Range of emotions include anger to euphoria.
* Cognitive function usually not affected.
* Emotional lability may negatively affect progress of disease.

Symptoms: Bowel & Bladder
* Symptoms depend on where sclerotic plaque is located.
* Spastic bladder indicates lesion above S2 resulting in small capacity. This yields frequency, urgency, dribbling or incontinence.
* Flaccid bladder results form disruption of reflex arc resulting in loss of sensation or desire to void.
o Crede Method
* Bowel symptoms are usually constipation.

Multiple Sclerosis Drug Therapy
Agent
Corticosteroid (ACTH, Prednisone)
Immunomodulators (B-interferon,Betaseron, Copaxone)
Immunosuppresants (Novantrone)
Cholinergics (Urecholine, Prostigmin)
Anticholinergics (Pro-bathine, Ditropan)
Muscle Relaxants (Valium, baclofen, Zanaflex)

Target Symptom
Exacerbations
Urinary retention; flaccid bladder
Urinary freq. and urgency; spastic bladder
Spasticity

Nursing Care Planning
* Impaired physical mobility
* Self-care deficit
* Impaired skin integrity; risk/actual
* Impaired elimination; urinary, bowel
* Sexual dysfunction
* Interrupted family process

Myasthenia Gravis
Definition & Pathophysiology
* Autoimmune disease of the neuromuscular junction in which antibodies attack acetylcholine (ACh) receptors.
* ACh is prevented from attaching and stimulating muscle contraction
* 90% of patients have ocular manifestations (ocular myasthenia)
* Generalized myasthenia affecting trunk and limbs less common.
* 15% of patients have abnormal thymus glands.
* No other neural disorders accompany MG.
o no sensory loss, reflexes are normal, atrophy is rare.

Myasthenic Crisis
An acute exacerbation of muscle weakness
o triggered by infection, surgery, stress, overdose or or inadequate management of medications
o If muscle weakness affects swallowing and breathing: aspiration, respiratory insufficiency, and infection may result.

Myasthenia Gravis: Symptoms
* Weakness of skeletal muscle
o Muscles are strongest in morning
o Evening: muscle weakness is prominent
* Impaired facial mobility and expression
* Prolonged speech resulting in fading voice
* Possible difficulty in chewing, swallowing
* Proximal muscles of neck, shoulder and hip may be affected
* Course of the disease is variable It most commonly affects young adult women (under 40) and older men (over 60), but it can occur at any age.
* CRISIS: myasthenic crisis is weakness affecting the muscles that control breathing, creating a medical emergency and requiring a respirator for assisted ventilation
Diagnosis
* Diagnosis may be made by H&P.
* Blood tests:
o antibodies to ACh in 90%
* EMG:
o show decreasing response to repeated stimulation of hand muscles
* Tensilon test: shows improved contractility with IV injection of medication.
* NEURO ASSESSMENT: MG expected findings: impairment of eye movements or muscle weakness without any changes in the individual's ability to feel things

Treatment
* Drug therapy – anticholinesterase agents, corticosteroids, immunosuppressants
* Surgical therapy - Thymectomy
* Plasmapheresis
* Intravenous IgG
* Rest!

Huntington’s Disease
* Autosomal dominant genetic disorder
* Deficiency in the acetylcholine and GABA results in excess dopamine.
* Symptoms:
o Clinically opposite of Parkinson’s
o Chorea, intellectual decline, emotional lability
http://www.youtube.com/watch?v=UKbC1jRjs3M
http://www.youtube.com/watch?v=gBtji6Ibbso

Huntington’s Disease: Symptoms
* Abnormal & excessive involuntary movements (chorea)
* Writhing and twisting movements of face, limbs, body.
* Facial involvement affects speech, chewing, swallowing causing aspiration, malnutrition.
* Gait deteriorates with loss of mobility.
* Mental function declines including intellect, emotional lability and psychotic behavior.

Huntington’s Disease Diagnosis
* By symptoms
* By genetic testing as an adult: IMPLICATIONS??
* By prenatal screening

Treatment
* Palliation, symptomatic
* Neuroleptics
* Antidepressants
* Chorea antagonists (Klonopin)
* Fetal tissue transplant (experimental)

Parkinson’s Disease
Definition & Pathophysiology
* Disease of the basal ganglia
* Substantia Nigra:
o Degeneration of dopamine producing neurons
o Disrupts normal balance between dopamine and acetylcholine in the basal ganglia
* Dopamine is essential for normal function of extrapyramidal motor system which controls
o posture
o support
o voluntary motion

Dopaminergic Synaptic Activity
Good to know: These neurotransmitters and functions highly related to “reward” and to psych drug actions
Symptoms
* Onset:
o gradual, insidious, and prolonged
* Classic triad:
o tremor, rigidity and bradykinesia
* Initially:
o may involve one side of the body with mild tremor, slight limp and decreased arm swing
* With progression:
o classic shuffling and propulsive gait with flexed arms and loss of postural reflexes develops
o Slowed reflexes include decrease blinking, drooling, and masked facies

Parkinson’s Presentation

Parkinson’s Disease

A firm diagnosis of Parkinson’s is made only if at least two of the classic triad symptoms are present.

Diagnosis Confirmed: improvement of symptoms once anti-parkinsonian drugs initiated

Parkinson’s Disease: Treatment

Drug
* Dopaminergic (levodopa, sinemet, parlodel, etc.)
* Anticholinergic (artane, cogentin, symmetrel, etc.)
* Antihistamine * MAO inhibitor
* COMT inhibitor’s (comtan, tasmar)

Symptom relieved
* Bradykinesia, tremor, rigidity
* Tremor
* Tremor, rigidity
* Bradykinesia, tremor, rigidity
* Slows breakdown of levodopa

Parkinson’s Disease: Treatment Surgical
* Ablation
* Deep brain stimulation
* Fetal tissue transplant

Nutritional
* Hi fiber
* Mechanical soft
* Frequent small meals
* Lo-protein in HS

Nursing Care Planning
* Impaired physical mobility
o Teaching necessary
* Dressing/grooming self-care deficit
* Impaired skin integrity-risk/actual
* Impaired urinary elimination
* Sexual dysfunction
* Interrupted family process

Seizure Disorders & Epilepsy
Definitions
Seizure:
o paroxysmal, uncontrolled electrical discharge of neurons in the brain that interrupts normal function
Epilepsy:
o spontaneously recurring seizures caused by a chronic underlying condition

Question:
* The population with the highest prevalence of new-onset epilepsy is:

A: over the age of 60
B: children under 5
C: adolescents between 12-18 yrs
D: middle-aged men

Seizure Classification
http://www.youtube.com/watch?v=CDccChHrgRA

Seizure Disorders & Epilepsy: Classifying Seizures
Two major classes:
* Generalized
* Partial

Depending on type, phases may include:
* Prodromal phase- signs & activity preceeding seizure
* Aural phase- sensory warning
* Ictal phase- full seizure
* Postictal phase- recovery

Seizure Disorders & Epilepsy: Generalized
* Absence
* Myoclonic
* Clonic
* Tonic
* Tonic-Clonic
* Atonic

Seizure Disorders & Epilepsy: Partial Seizures
* Simple: no impairment of consciousness
o Symptoms: motor, somatosensory, autonomic, psychic
* Complex: impairment of consciousness
o Simple with progression to LOC/impairment
+ Symptoms: no other features, simple partial seizure features, automatisms
o Impairment of consciousness at onset
+ Symptoms: no other features, simple partial seizure features, automatisms

Seizure Disorders & Epilepsy: Status Epilepticus
* Medical emergency
* Seizure repeated continuously
o Tonic clonic: hypoxia could develop if muscle contraction is lengthened. Also: hypoglycemia, acidosis, hypothermia, brain damage, death
+ IV administration of antiepileptics
+ Maintain airway patency

Seizure Disorders & Epilepsy: Diagnostic Studies
* Most useful tool:
o a reliable and accurate description of the event, and the patient’s health history
* PE
* Electroencephalography (EEG):
o only useful when it shows abnormalities
* Labs:
o CBC, U/A, BMP
* PET scan, CT, MRI, MRA, MRS

Drug Therapy
* Can be helpful in preventing ongoing seizures following a head injury and should be first priority to decrease likelihood of second seizure

Seizure Disorders & Epilepsy Drug Therapy for Tonic-Clonic and Partial Seizures
* Carbamezepine/ Tegretol
* Divalproex/ Depakote
* Gabapentin/ Neurontin
* Lamotrigine/ Lamictal
* Levetiracetam/ Keppra
* Phenytoin/ Dilantin
* Tiagabine/ Gabitril
* Topiramate/ Topamax
* Valproic Acid/ Depakene
* Felbamate/ Felbatol *
* Phenobarbitol**

*Felbatol has been associated with aplastic anemia
**Phenobarbitol is a barbituate
Seizure Disorders & Epilepsy
Drug Therapy for:
Absence, Akinetic, & Myoclonic Seizure
* Clonazepam/ Klonopin
* Divalproex/ Depakote
* Valproic Acid/ Depakene
* Ethosuximide/ Zarontin
* Phenobarbitol

Seizure Disorders & Epilepsy Toxic Side Effects
* Diplopia
* Drowsiness
* Ataxia
* Mental slowing
assess for dose related toxicity including nystagmus, hand and gait coordination, cognitive function, general alertness
Seizure Disorders & Epilepsy
Idiopathic Side Effects
* Skin rash
* Gingival hyperplasia (dilantin)
* Bone marrow & blood dyscrasia
* Abnormal liver function
* Abnormal kidney function

Seizure Disorders & Epilepsy: Nursing Care

* Assure oxygen and suction equipment at bedside
o http://www.youtube.com/watch?v=H2vH1igOoh0
* Safety precautions in active stage
o Support/ protect head
o Turn to side
o Lossen constricted clothing
o Ease to floor
* Time seizure, record details of seizure and post-ictal phase
* Patient teaching:
o importance of good seizure control using medication as ordered
o Medical alert bracelet
o Avoid decreased sleep, increased EtOH, fatigue
o Regular meals/ snacks

A Potpourri of Neurological Disorders.ppt