Chapter 17 Disorders of the Central and Peripheral Nervous Systems and the Neuromuscular Junction Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. Brain Trauma Traumatic brain injury A traumatic insult to the brain possibly producing physical, intellectual, emotional, social, and vocational changes Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 2 At-Risk Individuals Infants 6 months to 2 years School-age children Adolescents and young adults 15 to 35 years of age People more than 70 years of age Men 1.5 times as likely to sustain a TBI Persons living in high-crime areas Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 3 Brain Trauma Blunt (closed, nonmissile) trauma Head strikes hard surface or a rapidly moving object strikes the head The dura remains intact; brain tissues not exposed to the environment Causes focal (local) or diffuse (general) brain injuries Open (penetrating, missile) trauma Injury breaks the dura and exposes the cranial contents to the environment Causes primarily focal injuries Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 4 Brain Trauma Compound fractures Basilar skull fracture Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 5 Brain Trauma Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 6 Causes of Brain Injury Falls 28% Motor vehicle crashes 20% Moving objects or moving against stationary objects 19% Assault 11% Sports-related events Blasts (military active duty personnel) Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 7 Severity of Brain Injury 75% to 90% of head injuries not severe Focal brain injury and diffuse axonal injury (DAI) each account for half of all injuries Focal brain injury accounts for more than two thirds of head injury deaths; DAI less than one third of deaths DAI accounts for the greatest number of severely disabled survivors Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 8 Severity of Brain Injury Hallmark of severe brain injury Loss of consciousness for six or more hours Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 9 Focal Brain Injury Observable brain lesion Cerebral edema Coup injury Injury directly below the point of impact Contrecoup Injury on the pole opposite the site of impact Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 10 Focal Brain Injury Force of impact typically produces contusions Contusions can cause: Extradural (epidural) hemorrhage or hematoma Subdural hematoma Intracerebral hematoma Clinical manifestations of contusion Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 11 Extradural Hematoma 85% arterial bleeding 15% meningeal vein or dural sinus injury 90% have a skull fracture The temporal fossa is the most common site of extradural hematoma caused by injury to the middle meningeal artery or vein Clinical manifestations of hematoma Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 12 Subdural Hematoma 10% to 20% of persons with traumatic brain injury MVAs are the most common cause 50% of subdural hematomas associated with skull fractures Falls (older adults, substance abuse) Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 13 Subdural Hematoma Acute Develops within 48 hours Often located at the top of the skull Chronic Develops over weeks to months • Older adults • Alcohol abuse • 80% complain of chronic headaches and have tenderness at site of injury Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 14 Intracerebral Hemorrhage Associated with MVA and falls Intracerebral hemorrhage and resultant hematoma acts as an expanding mass Increased ICP and compression of brain tissues with resultant edema Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 15 Diffuse Brain Injury DAI Shaking, inertial effect Acceleration/deceleration Axonal damage • Shearing, tearing, or stretching of nerve fibers Severity corresponds to the amount of shearing force applied to the brain and brainstem (mild, moderate, severe) Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 16 Diffuse Brain Injury Concussions Mild concussion Classical concussion Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 17 Mild Concussion Temporary axonal disturbances causing attention and memory deficits but no loss of consciousness I—confusion, disorientation, and momentary amnesia II—momentary confusion and retrograde amnesia III—confusion with retrograde and anterograde amnesia Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 18 Classic Cerebral Concussion Grade IV Disconnection of cerebral systems from the brainstem and reticular activating system Physiologic and neurologic dysfunction without substantial anatomic disruption Loss of consciousness (<6 hours) Anterograde and retrograde amnesia Uncomplicated (no focal injury) Complicated (focal injury) Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 19 Postconcussive Syndrome Headache, cognitive impairments, psychologic and somatic complaints, cranial nerve signs and symptoms Treatment Reassurance and symptomatic relief Close observation for 24 hours by a reliable individual so immediate intervention can be obtained if delayed effects become severe Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 20 Spinal Cord Trauma Commonly occurs from vertebral injuries Traumatic injury of vertebral and neural tissues due to compressing, pulling, or shearing forces Most common locations: cervical (1, 2, 4-7), and T1-L2 lumbar vertebrae Simple fracture, compressed fracture, and comminuted fracture Locations reflect most mobile portions of vertebral column and the locations where the spinal cord occupies most of the vertebral canal Primary vs. secondary injury Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 21 Spinal Cord Trauma Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 22 Spinal Cord Trauma Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 23 Spinal Cord Trauma Spinal shock Normal activity of the spinal cord ceases at and below the level of injury; sites lack continuous nervous discharges from the brain Complete loss of reflex function (skeletal, bladder, bowel, sexual; thermal control; autonomic control) Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 24 Spinal Cord Trauma Neurogenic shock Loss of sympathetic outflow • Vasodilation • Hypotension • Bradycardia • Hypothermia Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 25 Spinal Cord Trauma Paraplegia Quadriplegia Autonomic hyperreflexia (dysreflexia) Massive, uncompensated cardiovascular response to stimulation of the sympathetic nervous system Stimulation of the sensory receptors below the level of the cord lesion Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 26 Spinal Cord Trauma Chemical and metabolic changes in tissues Release of toxic excitatory amino acids, accumulation of endogenous opiates, lipid hydrolysis with production of active metabolites, and local free radical release Produce further ischemia, vascular damage, and necrosis of tissues Necrosis consumes 40% of cross-sectional cord within 4 hours of trauma and 70% within 24 hours Cord swelling increases degree of dysfunction • Distinguishing functions to be lost permanently from those that are impaired temporarily becomes difficult • In the cervical region, cord swelling may be life threatening Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 27 Autonomic Hyperreflexia Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 28 Degenerative Disorders of the Spine Degenerative disk disease (DDD) Spondylolysis Spondylolisthesis Spinal stenosis Low back pain Herniated intervertebral disk Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 29 Cerebrovascular Disorders Cerebrovascular accidents (CVAs) Thrombotic stroke • Arterial occlusions caused by thrombi formed in arteries supplying the brain or in intracranial vessels • Transient ischemic attacks Embolic stroke • Fragments that break from a thrombus formed outside the brain Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 30 Cerebrovascular Disorders Hemorrhagic stroke (ICH) Lacunar stroke Cerebral infarction Cerebral hemorrhage Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 31 Cerebrovascular Disorders Intracranial aneurysm Saccular (berry) aneurysms Fusiform (giant) aneurysms Mycotic aneurysms Traumatic aneurysms • Dissecting aneurysms Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 32 Intracranial Aneurysm Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 33 Cerebrovascular Disorders Vascular malformations Cavernous angiomas Capillary telangiectasis Venous angioma Arteriovenous malformation Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 34 Cerebrovascular Disorders Subarachnoid hemorrhage Manifestations Blood escapes from defective or injured vasculature into the subarachnoid space Kernig sign Brudzinski sign Risk factors Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 35 Headache Migraine headache Trigger factors Aura Cluster headache Several attacks can occur during the day for days followed by a long period of spontaneous remission Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 36 Headache Chronic paroxysmal hemicrania Cluster-type headache that occurs with more daily frequency but with shorter duration Tension-type headache Mild to moderate bilateral headache with a sensation of a tight band or pressure around the head Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 37 Central Nervous System Tumors Cranial tumors Primary intracerebral tumors (gliomas) • Astrocytoma • Oligodendroglioma • Ependymoma Primary extracerebral tumors • Meningioma • Nerve sheath tumors • Metastatic carcinoma Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 38 Central Nervous System Tumors Spinal cord tumors Intramedullary tumors Extramedullary tumors • Intradural • Extradural Manifestations • Compressive syndrome • Irritative syndrome • Syringomyelic syndrome Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 39 Spinal Cord Tumors Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 40 Infection and Inflammation of the CNS Meningitis Bacterial meningitis Aseptic (viral, nonpurulent, lymphocytic) meningitis Fungal meningitis Tubercular (TB) meningitis Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 41 Infection and Inflammation of the CNS Suppurative cerebral masses Cerebral abscess Spinal cord abscess • Intramedullary spinal cord abscess Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 42 Infection and Inflammation of the CNS Encephalitis Acute febrile illness, usually of viral origin with nervous system involvement Most common forms of encephalitis are caused by arthropod-borne viruses and herpes simplex virus Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 43 Neurologic Complications of AIDS HIV-associated cognitive dysfunction HIV myelopathy HIV neuropathy Aseptic viral meningitis Opportunistic infections Cytomegalovirus infections Parasitic infection CNS neoplasms Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 44 HIV Dementia Affects adults and children Insidious and unpredictable course Impaired concentration, short-term memory, retrieval Generalized cognitive system deficits Occur later Psychomotor slowing Decreased speech spontaneity and fluency Progressive loss of balance, ataxia, spastic paraparesis or paralysis, and generalized hyperreflexia Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 45 Lyme Disease Tick-borne spirochete bacterial infection Borrelia burgdorferi Progression Introduced by tick bite Incubates 3 to 32 days Migrates to skin, lymph nodes, and other body systems Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 46 Lyme Disease Stages 1 Bull's eye rash 2 Cardiac and neurological manifestations 3 MS, enhanced neurological manifestations Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 47 Demyelinating Disorders Multiple sclerosis (MS) Progressive, inflammatory, demyelinating, autoimmune disorder of the CNS Degeneration of the myelin sheath in CNS neurons Types • Mixed (general) • Spinal • Cerebellar Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 48 Neurodegenerative Disorders Amyotrophic lateral sclerosis (ALS) Classic ALS—Lou Gehrig’s disease Diffusely affects upper and lower motor neurons of the cerebral cortex, brainstem, and spinal cord (corticospinal tracts and anterior roots) Progressive weakness leading to respiratory failure and death Person has normal intellectual and sensory function until death Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 49 Peripheral Nervous System Disorders Neuropathies Generalized symmetric polyneuropathies • Distal axonal polyneuropathy • Demyelinating polyneuropathy Generalized neuropathies • Sensory neuropathies Focal or multifocal neuropathies Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 50 Peripheral Nervous System Disorders Guillain-Barré syndrome Acquired inflammatory disease causing demyelination of the peripheral nerves with relative sparing of axons Acute onset, ascending motor paralysis Humoral and cellular immunologic reaction Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 51 Peripheral Nervous System Disorders Radiculopathies Radiculitis • Inflammation of the spinal nerve roots Radicular pain Plexus injures Involves the nerve plexus distal to the spinal roots but proximal to the formation of the peripheral nerves Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 52 Neuromuscular Junction Disorders Myasthenia gravis Chronic autoimmune disease IgG antibody produced against acetylcholine receptors (antiacetylcholine receptor antibodies) Weakness and fatigue of muscles of the eyes and the throat, causing diplopia, difficulty chewing, talking, swallowing Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 53 Neuromuscular Junction Disorders Myasthenia gravis Classification • Neonatal myasthenia • Congenital myasthenia • Juvenile myasthenia • Ocular myasthenia • Generalized autoimmune myasthenia Myasthenia crisis Cholinergic crisis Mosby items and derived items © 2010, 2006 by Mosby, Inc., an affiliate of Elsevier Inc. 54