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Huntington’s Chorea

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. Hydrocephalus. Pronounced (high-droh- SEFF -ah-lus) Defined

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Huntington’s Chorea

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  1. 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

  2. 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

  4. 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

  5. Primary Intracranial Tumors • 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

  6. Metastatic Intracranial Tumors (Secondary) • 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

  7. 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

  9. 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

  10. 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

  11. 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

  12. 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

  13. 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

  14. 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

  15. Post Polio Syndrome • Pronounced • (POST POH-lee-oh SIN-drom) • Defined • Progressive weakness occurring at least 30 years after the initial poliomyelitis attack

  16. 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

  17. 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

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

  19. 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

  20. 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

  21. 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

  22. 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

  23. 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

  24. 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

  25. Quadriplegia • 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

  26. 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

  27. 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

  28. DIAGNOSTIC TECHNIQUES, TREATMENTS, AND PROCEDURES Nervous System

  29. Diagnostic Techniques, Treatments, and Procedures • 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

  30. Diagnostic Techniques, Treatments, and Procedures • 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

  31. 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

  32. Diagnostic Techniques, Treatments, and Procedures • 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

  33. Diagnostic Techniques,Treatments, and Procedures • 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

  34. Diagnostic Techniques, Treatments, and Procedures • Chordotomy • Neurosurgical procedure for pain control accomplished through a laminectomy • Surgical interference of pathways within the spinal cord that control pain

  35. Diagnostic Techniques,Treatments, and Procedures • 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

  36. Diagnostic Techniques, Treatments, and Procedures • 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

  37. Diagnostic Techniques, Treatments, and Procedures • 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

  38. Diagnostic Techniques, Treatments, and Procedures • Laminectomy • Surgical removal of the bony arches from one or more vertebrae in order to relieve pressure from the spinal cord

  39. Diagnostic Techniques, Treatments, and Procedures • Electromyography (EMG) • Process of recording electrical activity of muscle • Insert small needle into the muscle, deliver small current that stimulates the muscle

  40. Diagnostic Techniques, Treatments, and Procedures • 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

  41. Diagnostic Techniques, Treatments, and Procedures • 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

  42. Diagnostic Techniques, Treatments, and Procedures • 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

  43. Diagnostic Techniques, Treatments, and Procedures • 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)

  44. Diagnostic Techniques, Treatments, and Procedures • 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

  45. Diagnostic Techniques, Treatments, and Procedures • 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

  46. Diagnostic Techniques, Treatments, and Procedures • 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

  47. Diagnostic Techniques, Treatments, and Procedures • 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

  48. Diagnostic Techniques, Treatments, and Procedures • 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

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