Huntington’s Chorea

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Huntington’s Chorea• Pronounced

– (HUNT-ing-tonz koh-REE-ah)• Defined

– Inherited neurological disease characterized by rapid, jerky, involuntary movements and increased dementia• Progressive, degenerative disease

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Hydrocephalus• Pronounced

– (high-droh-SEFF-ah-lus)• Defined

– Abnormal increase of cerebrospinal fluid in the brain that causes the ventricles of the brain to dilate• Results in increased head circumference in infant

with open fontanel• Congenital disorder

3

Intracranial Tumors• Pronounced

– (in-trah-KRAY-nee-al TOO-morz)• Defined

– Tumors occurring in any structural region of the brain• May be malignant or benign• Classified as primary or secondary• Named according to the tissue from which they

originate

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Primary Intracranial Tumors• Pronounced

– (PRIGH-mah-ree in-trah-KRAY-nee-al TOO-morz)

• Defined– Tumors that arise from gliomas and the

meninges• Gliomas = malignant glial cells that are a support

for nerve tissue

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• Glioblastoma multiforme – Most rapidly growing of the gliomas

• Astrocytomas– Tend to invade surrounding structures

• Ependymomas – Occur more commonly in children and

adolescents– Usually encapsulated and benign

Primary Intracranial Tumors

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• Pronounced– (met-ah-STAT-ik in-trah-KRAY-nee-al TOO-

morz)• Defined

– Tumors occurring as a result of metastasis from a primary site such as the lung or breast

Metastatic Intracranial Tumors (Secondary)

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Meningitis (Acute Bacterial)

• Pronounced– (men-in-JYE-tis ah-KYOOT back-TEE-ree-al)

• Defined– Serious bacterial infection of the meninges

• Can have residual debilitating effects or even a fatal outcome

• Must be diagnosed and treated promptly with appropriate antibiotic therapy

8

Multiple Sclerosis (MS)• Pronounced

– (MULL-tih-pl SKLEH-roh-sis)• Defined

– Degenerative inflammatory disease of the central nervous system attacking the myelin sheath in the spinal cord and brain• Leaves area sclerosed (hardened) or scarred

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Myasthenia Gravis

• Pronounced– (my-ass-THEE-nee-ah GRAV-is)

• Defined– Chronic progressive neuromuscular disorder

• Causes skeletal muscle weakness (without atrophy) and fatigue

• Occurring at different levels of severity

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Narcolepsy• Pronounced

– (NAR-coh-lep-see)• Defined

– Rare syndrome of uncontrolled, sudden attacks of sleep

– Main features of narcolepsy are daytime sleepiness and cataplexy

– Sudden loss of muscle tone

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Neuroblastoma• Pronounced

– (noo-roh-blass-TOH-mah)• Defined

– Highly malignant tumor of the sympathetic nervous system• Most commonly occurs in the adrenal medulla with

early metastasis to liver, lungs, lymph nodes, and bone

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Parkinson’s Disease• Pronounced

– (PARK-in-sons dih-ZEEZ)• Defined

– Degenerative, slowly progressive deterioration of nerves in the brain stem’s motor system, characterized by a gradual onset of symptoms• Classic Symptoms: stooped posture with body

flexed forward, bowed head, shuffling gait, pill-rolling gestures, expressionless mask-like facial appearance

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Peripheral Neuritis• Pronounced

– (per-IF-er-al noo-RYE-tis)• Defined

– Inflammation of one or more peripheral nerves– Effects are dependent upon particular nerve

involved

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Poliomyelitis• Pronounced

– (poh-lee-oh-my-ell-EYE-tis)• Defined

– Infectious viral disease that affects the ability of spinal cord and brain motor neurons to receive stimulation• Virus enters through the upper respiratory tract• Muscles affected become paralyzed without the

motor nerve stimulation

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Post Polio Syndrome• Pronounced

– (POST POH-lee-oh SIN-drom)• Defined

– Progressive weakness occurring at least 30 years after the initial poliomyelitis attack

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Reye’s Syndrome• Pronounced

– (RISE SIN-drom)• Defined

– Acute brain encephalopathy along with fatty infiltration of the internal organs that may follow acute viral infections• Occurs in children under the age of 18; often with a

fatal result• Linked to aspirin administration during a viral

illness

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Shingles (Herpes Zoster)• Pronounced

– (SHING-lz) (HER-peez ZOSS-ter)• Defined

– Acute viral infection characterized by inflammation of the underlying spinal or cranial nerve pathway producing painful, vesicular eruptions on the skin along these nerve pathways• Seen mainly in adults

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Shingles

Image courtesy of Robert A. Silverman,M.D., Pediatric Dermatology,Georgetown University

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Skull Fracture (Depressed)• Pronounced

– (SKULL FRAK-chur, deh-PREST)• Defined

– Broken segment of the skull bone thrust into the brain as a result of a direct force, usually a blunt object

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Spina Bifida Cystica• Pronounced

– (SPY-nah BIFF-ih-dah SISS-tih-kah)• Defined

– Back portion of one or more vertebrae is not closed normally and a cyst protrudes through the opening in the back, usually at the level of the 5th lumbar or 1st sacral vertebrae• Congenital defect of the CNS

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Meningocele• Pronounced

– (men-IN-goh-seel)• Defined

– Cystlike sac covered with skin or a thin membrane protruding through the bony defect in the vertebrae containing meninges and CSF

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Meningomyelocele• Pronounced

– (men-in-goh-my-ELL-oh-seel)• Defined

– Cystlike sac covered with skin or a thin membrane protruding through the bony defect in the vertebrae that contains meninges, CSF, and spinal cord segments

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Spina Bifida Occulta• Pronounced

– (SPY-nah BIFF-ih-dah oh-KULL-tah)• Defined

– Congenital defect of the central nervous system in which the back portion of one or more vertebrae is not closed• A dimpling over the area may occur

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Paraplegia• Pronounced

– (pair-ah-PLEE-jee-ah)• Defined

– Paralysis of the lower extremities caused by severe injury to the spinal cord in the thoracic or lumbar region

– Results in loss of sensory and motor control below the level of the injury

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• Pronounced– (kwod-rih-PLEE-jee-ah)

• Defined– Paralysis of the trunk, legs, and pelvic organs

with partial or total paralysis in the upper extremities caused by severe injury to the spinal cord between the 5th and 8th cervical vertebrae• The higher the trauma, the more debilitating the

motor and sensory impairments

Quadriplegia

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Tay-Sachs Disease• Pronounced

– (TAY SACKS dih-ZEEZ)• Defined

– Congenital disorder caused by altered lipid metabolism, resulting from an enzyme deficiency• Accumulation of this type of lipid occurs in the

brain, leading to progressive neurological deterioration with both physical and mental retardation

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Trigeminal Neuralgia(Tic Douloureux)

• Pronounced– (try-JEM-ih-nal noo-RAL-jee-ah),– (tik DOO-loh-roo)

• Defined– Short periods of severe unilateral pain which

radiates along the fifth cranial nerve (trigeminal nerve)• Heat, chewing, or touching of the affected area

activates the pain

DIAGNOSTIC TECHNIQUES, TREATMENTS, AND

PROCEDURES

Nervous System

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• Babinski’s Reflex– Reflex tested by stroking the sole of the foot,

beginning at mid-heel and moving upward and lateral to the toes.• Positive Babinski’s occurs when there is

dorsiflexion of the great toe and fanning of the other toes

Diagnostic Techniques, Treatments, and Procedures

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• Brain scan– Nuclear counter scanning of cranial contents

two hours after an intravenous injection of radioisotopes • Isotopes concentrate in abnormal tissue of brain,

indicating a pathological process

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Diagnostic Techniques,Treatments, and Procedures

• Cerebral Angiography– Visualization of the cerebral vascular system

via x-ray after injection of a radiopaque contrast medium into an arterial blood vessel • May use carotid, femoral, or brachial artery

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• Cerebrospinal fluid analysis – Laboratory analysis of cerebrospinal fluid– Obtained from a lumbar puncture for the

presence of bacteria, blood, malignant cells, and amount of protein and glucose present

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• CT scan of the brain– Analysis of a three-dimensional view of brain

tissue obtained as X-ray beams pass through successive horizontal layers of the brain• CT = computerized tomography• Images look down through the top of the head

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• Chordotomy– Neurosurgical procedure for pain control

accomplished through a laminectomy • Surgical interference of pathways within the spinal

cord that control pain

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• Cisternal puncture– Insertion of a short, beveled spinal needle into

the cisterna magna in order to drain CSF or to obtain a CSF specimen• Cisterna magna = shallow reservoir of CSF

between the medulla and the cerebellum

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• Craniotomy– Surgical procedure that makes an opening

into the skull• Echoencephalography

– Measurement of electrical activity produced by the brain and recorded through electrodes placed on the scalp

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• Electroencephalography (EEG)– Measurement of electrical activity in the brain

and recorded through electrodes – Sleep-deprived EEG

• Individual deprived of sleep for 24 hours before test– Ambulatory EEG

• Provides prolonged readings of electrical activity of brain over a 24-hour period of time, while person is awake or asleep

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• Laminectomy– Surgical removal of the bony arches from one

or more vertebrae in order to relieve pressure from the spinal cord


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• Electromyography (EMG)– Process of recording electrical activity of

muscle– Insert small needle into the muscle, deliver

small current that stimulates the muscle

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• Lumbar puncture– Insertion of a hollow needle and stylet into

subarachnoid space between third and fourth lumbar vertebrae below level of the spinal cord

– Performed under strict aseptic technique

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• Magnetic Resonance Imaging (MRI)– Noninvasive scanning procedure that provides

visualization of fluid, soft tissue, and bony structures without the use of radiation• Provides far more preciseness and accuracy than

most diagnostic tools– Not limited to scans of the brain

• May also be used to examine the abdomen, chest, joints, nervous system, pelvis, and spinal column

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• Myelography– Introduction of contrast medium into the

lumbar subarachnoid space through a lumbar puncture in order to visualize the spinal cord and vertebral canal through x-ray examination

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• Neurectomy– Neurosurgical procedure to relieve pain in a

localized or small area by incision of cranial or peripheral nerves

• Pneumoencephalography– Process used to radiographically visualize one

of the ventricles or fluid occupying spaces in the central nervous system (CNS)

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• Polysomnogram (PSG)– Sleep study or sleep test that evaluates

physical factors affecting sleep• Physical activity and level of sleep are monitored

by a technician while the patient sleeps– Useful in evaluating sleep disorders such as

sleep apnea, sleep walking, night terrors, restless leg syndrome, insomnia, and narcolepsy

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• Positron Emission Tomography (PET Scan)– Computerized radiographic images of various

body structures produced when radioactive substances are inhaled or injected

• Romberg test– Examination used to evaluate cerebellar

function and balance

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• Stereotaxic Neurosurgery– Neurosurgery on a precise location of an area

within the brain that controls specific function(s) • May involve destruction of brain tissue with various

agents such as heat, cold, and sclerosing or corrosive fluids

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• Sympathectomy– Surgical procedure used to interrupt a portion

of the sympathetic nerve pathway, for the purpose of relieving chronic pain

• Trichotomy– Through a craniotomy, the anterolateral

pathway in the brain stem is surgically divided in an attempt to relieve pain

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• Transcutaneous Electrical Nerve Stimulation (TENS)– Form of cutaneous stimulation for pain relief

that supplies electrical impulses to the nerve endings of a nerve close to the pain site

Huntington’s Chorea

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