Ch 70 Oncologic or Degenerative Neurologic Disorders

Signs of Huntington disease?

-Motor dysfunction (chorea) -cognitive impairment (attention and emotion recognition) -behavioral features (apathy, blunt affect) also: tics, slurred speech, strange gait

Management of ALS

-No cure, treatment or prevention. -Riluzole gives extended survival time, by 3-6 months -Respiratory support exercise and mobility program

Treatment options for brain tumor

-Surgery to remove as much tumor as possible without increasing the neurologic deficit (paralysis, blindness), or to relieve symptoms by partial removal (decompression) -Radiation therapy -Chemotherapy: greatest challenge is that the blood-brain barrier prevents drugs from getting to the tumor in effective doses without causing systemic toxicity -Pharmacologic therapy

What are the three classifications for spinal cord tumors?

-intramedullary lesions (within the spinal cord) -extramedullary-intradural lesions (within or under the spinal dura) -extramedullary-extradural lesions (outside the dural membrane)

Each child of a parent with Huntington disease has a what % risk of inheriting the disorder?

50% risk

A patient with a brain tumor wouldn't be prescribed Coumadin, an anticoagulant, because why?

Although deep vein thrombosis and pulmonary embolism occur in about 15% of patients and cause significant morbidity, anticoagulants are not prescribed due to the risk for CNS hemorrhage.

What is ALS?

Amyotrophic Lateral Sclerosis (ALS) is a disease of unknown cause in which there is a loss of motor neurons (nerve cells controlling muscles) in the anterior horns of the spinal cord and the motor nuclei of the lower brainstem. It is often referred to as Lou Gehrig disease.

Describe herniation of a cervical intervertebral disc

C5-C6 or C6-C7 interspaces and compresses a unilateral nerve root, can cause pain or stiffness around neck, shoulders, scapulae region, pain, and paresthesia

A client with a brain tumor is experiencing changes in cognition that require the nurse to reorient the client frequently. When performing this task, which devices would be appropriate for the nurse to use? Select all that apply.

Client's clothing Picture of the client's family Clock Calendar words would not be as helpful as items that are familiar to the client.

How is a diagnosis of Huntington disease made?

Clinical presentation of symptoms, positive family history, genetic marker CAG repeats with HTT (Huntington gene)

Bone density testing will be completed for the client with post-polio syndrome. The nurse teaches the client bone density testing is used to identify what potential complication?

Low bone mass & osteoporosis

What is most commonly used to diagnose brain and spinal tumors?

MRI scan

What is the most common type of brain tumor?

Glioma, most common glioma is astrocytomas

What is the treatment for Parkinson's disease?

Levodopa= its converted to dopamine in the basal ganglia, producing symptom relief. It works best within 1-2 years of starting the drug, but within 5-10 years pts have a bad response to the drug (dyskinesias)

How are ALS diagnosis made?

No tests are specific to diagnosing ALS, so it is diagnosed on the basis of symptoms.

Describe management regarding movement for someone with herniation of a lumbar disc

Pain gets worse when bending, lifting, straining, sneezing or cough because this increases; and usually is relieved by bed rest. However, Bed rest is discouraged because it may weaken muscles, but activities that exacerbate pain should be avoided.

What is a potential complication post op with a pt who has a tumor in the cervical area of the spinal cord?

Respiratory compromise due to postoperative edema

What are the most common causes of death for those with muscular dystrophies?

Respiratory infection & cardiac failure

How is a herniated disc diagnosed?

The MRI scan has become the diagnostic tool of choice for localizing even small disc protrusions, particularly for lumbar spine disease. If the clinical symptoms are not consistent with the pathology seen on MRI, CT scanning and myelography are performed.

Medical management for those with muscular dystrophies?

The goal is supportive management to keep the pt active & functioning as normally as possible & minimize functional deterioration. To help prevent spinal deformity, the patient is fitted with an orthotic jacket to improve sitting stability and reduce trunk deformity. Weakness of the facial muscles makes it difficult to attend to dental hygiene and to speak clearly, and impairs the ability to swallow safely

Describe post-polio syndrome (PPS)

exact cause of PPS not known, but may be something to do w/ aging or muscle overuse. Those who survived polio may experience this 30-40 years after exposure (w/ or w/o polio symptoms at the time)

Signs of ALS

fatigue, progressive muscle weakness, cramps, fasciculations (twitching), lack of coordination, Spasticity later difficulty swallowing, coughing, speaking etc

How is Parkinson's disease diagnosed?

from the patient's history and the presence of two of the four cardinal manifestations: tremor, rigidity, bradykinesia, and postural changes.

What is chorea?

rapid, jerky, involuntary, purposeless movements of the extremities or facial muscles, including facial grimacing

How does deep brain stimulation work (DBS)?

Treatment for Parkinson's disease -surgical implantation of electrode into the brain in either the globus pallidus or subthalamic nucleus. Stimulation of these areas may increase dopamine release or block anticholinergic release, thereby improving tremor and rigidity.

What are cerebral metastases?

tumors that originate from outside the CNS (Brain and spinal cord) and spread to it via the blood stream or directly invade from neighbouring tissues. More common than primary brain tumor

Signs of increasing ICP (which are also signs of brain tumor)

Headache, vomiting w/ or w/o nausea, papilledema (swelling of the optic nerve), seizures

Describe Huntington's disease

Hereditary disorder transmitted as an autosomal dominant gene. Causes cells of basal ganglia (involved in the control of movement) and cerebral cortex to die prematurely.

What does a cervical collar do?

Limits neck motion and immobilizes spine to prevent injury. If prescribed its normally for about 6 weeks

Describe herniation of a lumbar disc

Most lumbar disc herniations occur at the L5-S1 region. produces low back pain accompanied by varying degrees of sensory and motor impairment. -low back pain, muscle spasm, sciatica (pain & tenderness radiating along sciatic nerve down thigh & leg)

Describe surgical management for herniated disc

Surgical excision of a herniated disc is performed if there is evidence of a progressing neurologic deficit (muscle weakness and atrophy, loss of sensory and motor function, loss of sphincter control) The goal of surgical treatment is to reduce the pressure on the nerve root to relieve pain and reverse neurologic deficits

What is a herniated disc?

When the nucleus pulpous (cushy center of disc) protrudes into the annulus (fibrous ring around disc) and causes pressure onto the spinal nerve root. A herniated disc with accompanying pain may occur in any portion of the spine: cervical, thoracic (rare), or lumbar. Signs depend of location & acute/chronic

What is dementia?

broad term for a syndrome characterized by a general decline in higher brain functioning, such as reasoning, with a pattern of eventual decline in ability to perform even basic activities of daily living, such as toileting and eating

Describe muscular dystrophies

group of incurable muscle disorders characterized by progressive weakening and wasting of the skeletal or voluntary muscles with 30 different types to date; most are inherited -signs: muscle stiffness or weakness, dysphagia, mental deficits, vision, or infertility

Describe Parkinson's Disease

slowly progressing neurodegenerative disorder that eventually leads to disability caused by a lack of dopamine in the substantia nigra of the basal ganglia

Medications for Huntington disease?

tetrabenazine for chorea (rapid, jerky, involuntary, purposeless movements) other meds Benzodiazepines, neuroleptics, SSRI's

Cardinal signs of Parkinson's disease

tremor, rigidity, bradykinesia (slowing of active movement), postural instability (strange posture & gait) other S/S: depression, dementia, orthostatic hypotension, drooling, etc