Problems of the Central Nervous System

EPILEPSY, SPINA BIFIDA, CEREBRAL PALSY

2009

SEIZURE DEFINED

• Episodes of abnormal sudden, excessive, uncontrolled electrical discharge of neurons within the brain

• May result in alteration in consciousness, motor or sensory ability and/or behavior

EPILEPSY

• Chronic disorder• Characterized by recurrent unprovoked

seizure activityCAUSE OF EPILEPSY • abnormal electrical neuronal activity• Imbalance of neurotransmitters: Gamma

aminobutyric acid (GABA)

CAUSE OF SEIZURES• Inherited• Cause is unknown (idiopathic)Can follow:• Birth trauma• Asphyxia during birth• Head injuries• Infectious disease• Toxicity (carbon monoxide and lead poisoning)• Fever• Drug/alcohol intoxication• Brain tumors

INTERNATIONAL CLASSIFICATION OF SEIZURES

• Classified by the part of the brain involved– PARTIAL SEIZURES beginning in one part of one cerebral

hemisphere• Complex partial seizures• Simple partial seizures

– GENERALIZED SEIZURES involving both cerebral hemispheres

– UNCLASSIFIED SEIZURES:• Unclassified• Idiopathic

PARTIAL SEIZURES

• Simple partial seizures • Complex partial seizures

PARTIAL SEIZURES

• SIMPLE PARTIAL SEIZURES:– Unilateral movement of extremity– Unusual sensations– Autonomic or psychic symptoms (heart rate,

flushing, epigastric discomfort)– May experience unusual or unpleasant sights,

sounds, odors, tastes (aura) occurring before seizure

– No loss of consciousness

COMPLEX PARTIAL

– Automatism: Person remains motionless or moves automatically, but inappropriately for time and place (eg: lip smacking, patting, picking at clothes)

– May experience excessive emotions of fear, anger, elation, or irritability

– No memory of episode, loss of consciousness, black out 1-3 min

GENERALIZED SEIZURES (previously GRAND MAL)

• TONIC-CLONIC SEIZURE– Lasts 2-5 min– Starts with tonic movements: stiffening or rigidity of the

muscles of arms and legs– Loss of consciousness– Clonic movements follow: rhythmic jerking of all

extremities– May bite tongue, incontinent of urine/feces– Followed by an hour of fatigue, confusion, lethargy

GENERALIZED SEIZURES CONTINUED

• Tonic seizures: – Abrupt increase in muscle tone– Loss of consciousness– Loss of autonomic signs for 30 sec to several minutes

• Clonic seizure: – Lasts several minutes– Muscle contraction and relaxation

GENERALIZED SEIZURES CONTINUED

• Absence seizure– Common in children– Runs in families– Brief (seconds) loss of consciousness– Blank starring, returning to normal– May occur frequently throughout day

• Myoclonic seizure:– Brief jerking/stiffening extremities– Singly or in groups– Lasts few seconds

GENERALIZED SEIZURES CONTINUED

• Atonic(akinetic)seizure– Sudden loss of muscle tone for a few seconds,

may fall– Followed by POSTICTAL(after seizure) confusion

POST-ICTAL STATE

• POST-ICTAL STATE: after seizure Pt is confused and hard to arouse, may sleep for hours

ASSESSMENT AND DIAGNOSTIC TESTS

• History• Identify causes• MRI• EEG• SPECT (single photon emission computed

tomography) – helps identify zone giving rise to seizures, can then be removed surgically

TREATMENT

• Remove or treat cause• Surgically remove/excise the part of the brain

that is causing the problem as long as it doesn’t produce neurologic deficits– Pt is alert

• Generator may be implanted under the clavicle: helps control the seizure

MEDICATIONS: antiepileptics (AED’s)

• Unknown why they work• Control seizures, do not cure• Starts with one med, dosage gradually increased looking for

SE, may add another drug• Therapeutic blood levels: Blood levels checked, absorption

varies among patients• Dosage changed with illness, weight changes, stress• Sudden withdrawal avoided• Lots of drug/drug interactions and drug/food interactions

SIDE EFFECTS OF DRUGS

• Allergic reaction (skin)• Acute toxicity (seen initially)• Chronic toxicity (seen later in therapy)• REACTIONS seen in an organ• Common SE of Dilantin: gingival hyperplasia

(swollen and tender gums)

ASSESSMENT DURING SEIZURE

• Assess for preseizure behavior, aura, loss of consciousness

• Assess seizure activity• Record the time the activity began• Assess for fecal or urinary incontinence• Assess for post seizure behavior (memory loss,

loss of consciousness, lethargy)

RECORD SEIZURE ACTIVITY

• Include onset time• Include focus of seizure (part of body involved)• Identify duration of seizure (time)• Identify change in respirations• Define progression of movement through body• Identify changes in neurological status• Describe post-ictal activity (duration, status,

behavior)

IMPLEMENTATION

• Gently lower the standing or sitting patient to the floor (supine)

• Turn the head to the side, hyperextend neck and pull jaw slightly forward

• Maintain patent airway• Have oxygen and suction available whenever a

patient indicates they have a history of seizures!!!• PLACE NOTHING IN THE MOUTH!

IMPLEMENTATION CONTINUED

• Do not restrain the patient• Remove dangerous objects the might injure

the patient• Loosen tight or restrictive clothing• Record seizure activity

TEACHING• Wear Medic Alert• Avoid stimulant drugs (caffeine) may cause a seizure

to break through the anticonvulsant medication• Avoid alcohol – can cause overdose of medications• Teach family/patient triggers to seizures:

hypoglycemia, fatigue, exhaustion, hormonal changes, illness, stress, alcohol, caffeine, constipation, hyperventilation, excessive activity

TEACHING CONTINUED

• High fiber diet/high fluid diet to prevent constipation caused by anticonvulsant meds

• Adequate rest, stress reduction, good diet• Anticonvulsant meds cause gingival hyperplasia:

need good dental care• Provide name of local Epilepsy Foundation• Assist in dealing with feelings

RESTRICTIONS until seizure free for 3 months to 1 year

• No driving motor vehicles• No operating heavy machinery• No working in potentially dangerous situations• No swimming, no water sports• Possibly no tub bathing• LENGTH OF RESTRICTION DEPENDS UPON

STATE REGULATIONS

MEDICATIONSDilantin (phenytoin)Therapeutic drug level 5-20 ug/dl• Cannot be withdrawn suddenly• Watch for toxicity (nystagmus, ataxia, dysarthria,

encephalopathy)• Do not combine with warfarin (Coumadin)

SIDE EFFECTS: • Lethargy, abnormal movements, Gingival hyperplasia, mental

confusion, cognitive changes

MEDICATIONS FOR STATUS EPILEPTICUS

Fosphenytoin (Cerebyx)Diazepam (Valium); give slowly, drug of choice

for status epilepticus, have ventilatory support available

Lorazepam (Ativan)

Given IV during status epilepticus

MEDICATIONS

• Ethosuximide (Zarontin): N & V, gastric distress, gradually withdraw

• Carbamazepine (Tegretol): expensive, watch effects on heart and lungs

• Valproate (Depakene): effects liver, causes tremors, alopecia

• Galbapentin (Neurotonin): dizziness, somnolence, wgt gain

MEDICATIONS

Clonazepam (Klonopin): drowsiness, palpitationsFelbamate (Felbatol): cognitive impairmentsLamotrigine (Lamictal): tremorLevetiracetam (Keppra): somnolenceOxacarbazepine (Trileptal): tremor, loss of

coordination

MEDICATIONS

• Phenobarbitol (Luminal): sedation• Primidone (mysoline): lethargy, impotence• Tiagabine (Gabitril): dizziness, buckling knees• Topiramate (Topamax): fatigue, anorexia, depression• Zonisamide (Zonegran): somnolence, agitation

STATUS EPILEPTICUS

• Series of generalized seizures tht occur without full recovery of consciousness between attacks

• Also includes continuous electrical seizures lasting at least 30 minutes even without impairment of consciousness

RISKS OF STATUS EPILEPTICUS

• Respiratory arrest at height of each seizure leading to hypoxia which can lead to brainb damage

FACTORS THAT TRIGGER STATUS EPILEPTICUS

• Withdrawal of seizure meds• Fever• Concurrent infection

STATUS EPILEPTICUS

MEDICAL EMERGENCY• cerbyx, Ativan, Valium IV• Oxygen & Airway device/intubation inserted

between seizures• Suction• Hydration IV using glucose for hypoglycemia

(high metabolic demand during seizure)

TREATMENT FOR STATUS EPILEPTICUS CONTINUED

• May have to anesthetize with short acting barbiturate to stop seizure

• Serum levels of anti- seizure meds• Cardiac/respiratory depression may be

lifethreatening• Cerebral edema can occur

CEREBRAL PALSY

CEREBRAL PALSY

• DEFINED AS:• A neurologic problem• Characterized by impaired movement and

posture• It involves a lack of motor control of voluntary

muscles• Comes from a lesion in the brain that occurred

prenatally, at birth or postnatally

CAUSE OF CP

• PRIMARY CAUSE: ANOXIA• SECONDARY CAUSE: INFECTION

CLINICAL MANIFESTATIONS

• Delayed gross motor development: universal symptom

• Abnormal motor performance• Alterations muscle tone: rigidity, stiffness• Abnormal postures: scissoring• Reflex abnormalities: persistance of primitive

infantile reflexes

ASSOCIATED DISABILITIES

• May have mental retardation• Common to have seizures• Attention deficit/hyperactivity• Sensory impairment

CLASSIFICATION

• Spastic: increased muscle tone• Dyskinetic (athetoid): abnormal writhing

movements, drooling, dysarthria, • Ataxic: difficulty with balance• Mixed: athetoid and spastic

DIAGNOSIS

• Know growth and development• Observe in newborn nursery• Early recognition important

TREATMENT

• TEAM APPROACHGoals:• Establish locomotion, communication, self-

help• To gain optimum function• To correct associated defects• To provide educational opportunities

MOBILIZING DEVICES

• Braces• Ambulation devices• Scooter boards• Wheeled go carts• Strollers• w/c

SURGERY

• Orthopedic• TAL (tendon achilles lengthening)• release of tight wrist and hip muscles

NURSING CARE

1. FEEDING: • Avoid semi-reclining• Place in flexed position • Gentle upward stroking of the neck2. DIET: high calorie diet3. MAINTAIN SKIN INTEGRITY4. PROMOTE SELF-CONCEPT

NURSING CARE CONTINUED

5. PROMOTE KNOWLEDGE 6. USE THE PARENTS AS BEST RESOURCE7. PROVIDE REST8. PREVENT/TREAT RESPIRATORY INFECTIONS9. DENTAL PROBLEMS10. PROVIDE PARENTAL SUPPORT

SPINA BIFIDA

SPINA BIFIDA

DEFINED: incomplete fusion of one or more vertebral laminae and defective development of the spinal cord

ETIOLOGY: • Environmental• Genetic• Vitamin deficiency

CATEGORIES

• MENINGOMYELOCELE OR MYELOMENINGOCELE

• MENINGOCELE• SPINA BIFIDA OCCULTA

MENINGOMYELOCELE (MMC)

• Most common• Involves the spinal cord• Have soft rounded protrusion• Sac containing meninges, portions of spinal

cord and nerve roots and CSF• Sac protrudes through defective vertebra• MASS DOES NOT TRANSILLUMINATE

MMC CONTINUED

• DEGREE OF NEUROLOGIC DYSFUNCTION• Flaccid lower extremities• Partial paralysis• Bowel and bladder incontinence• Deformities

MENINGOCELE

• Does not involve spinal cord• Sac like cyst contains meninges with CSF, no

neural tissue• MASS CAN BE TRANSILLUMINATED• LACK OF PARALYSIS

SPINA BIFIDA OCCULTA

• Common, usually asymptomatic• No external protrusion• Failure of spinous processes to join in the L5

to S1 area• Skin depression, dimple, birthmark, hair,

lipoma• May have bowel/bladder sphincter control

prblems

MANAGEMENT

Multidisciplinary approach:• Neurosurgeon• Orthopedic surgeon• GU• Psychologist• Educators• Social workers• Pediatric nurse clinician

NURSING MANAGEMENT AT BIRTH

• Place under overhead warmer• On abdomen• Keep sac moist

SAC CARE

PREVENT RUPTURE, DRYING AND INFECTION OF SAC

• Antibiotics IV• Prone• Skin care• Soaks on sac (NSS, bacitracin, silver nitrate)

COMPLICATIONS

• MENINGITIS• NEUROLOGIC INVOLVEMENT• HEAT AND FLUID LOSS• LEAKAGE OF SPINAL FLUID• DEFORMITIES

SURGICAL REPAIR

• Neurosurgeon and plastic surgeon• Excision of protrusion

POST-OP NURSING CARE

• Incubator/overhead warmer• Oxygen• Prone/some require head lowered• Plastic apron• NPO to clear fluids to full fluids• Daily head circumferences• Observe for hydrocephalus/meningitis/urinary

retention

CARE OF SITE AT HOME

• Wash the incision with peroxide and rinse with water to remove crusts

• Observe for drainage and call MD• Keep diaper below site • After healed, tub baths

LONG TERM PROBLEMS

ORTHOPEDIC ABNORMALITIES• Hip dislocations, club foot, scoliosis,

contractures in hips, knees, anklesNURSING CARE:• PROM, turning, check for reddened areas, pad

seats, cribs, soft toys, check toes, check braces

GENITOURINARY ABNORMALITIES

• Renal failure• UTI• Urinary retention• Urinary refluxTREATMENT: clean intermittent catherization

programEXPERIMENTAL: artificial sphincters, bladder

pacemaker

NURSING CARE BLADDER

• Change diapers q 2 hrs• Wash bottom with antibacterial soap• No rubber pants• A&D and Desitin• Drugs which cause urinary retention

BOWEL MANAGEMENT

• Bowel control depends upon innervation• Regular toilet habits• Prevention of constipation and impaction• Diet high in fiber and high fluids• BOWEL PROGRAM: 2-3 yrs of age• Rectalaid enemas, cathartic (senekot)

DEVELOPMENTAL CONSIDERATIONS

• Toddler: can bear down by coughing or blowing up balloons, bowel routine ritual

• Preschooler: asks why? Answer the truth• “It’s because of the opening on your back when you

were born. You can’t go to the bathroom like other people.”

• School age and Adolescent: do independently, concern – privacy, peer rejection

SEXUALITY

• Males: erection and ejaculation possible, most sterile from retrograde ejaculation

• Females: fertility well documented. 4-5% risk of having a MMC child. SEX ED IMPORTANT.

PSYCOSOCIAL:

Multifaceted:• Body image• Sexuality• Urinary problems• Bowel problems