1. Infectious Diseases Affecting the Nervous System
Chapter 19
Infectious Diseases Affecting the Nervous System

2. The Nervous System and Its Defenses
19.1 The Nervous System and Its Defenses A. Two component parts to the nervous system 1. CNS – Brain and spinal cord: made up of neurons, both surrounded by bone, encased with meninges 2. PNS: cranial and spinal nerves

3. The Nervous System and Its Defenses
B. Defenses of the Nervous System 1. Mainly structural A) Bony casings B) Cushion of CSF C) Blood-brain barrier D) Immunologically privileged site

4. Diseases of the Nervous System
19.3 Nervous System Diseases Caused by Microorganisms
A. Bacterial meningitis
1. Many different microorganisms can cause an infection
2. More serious forms caused by bacteria
A) Bacterial form is less common and most patients are children

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3. Typical symptoms: headache, painful or stiff neck, fever, and usually an increased number of white blood cells in the CSF 4. Causative agents:

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A) Neisseria meningitides 1) Gram-negative diplococci 2) Commonly known as meningococcus 3) Causes the most serious form of acute meningitis a) Greatly feared because it can result in shock and death within 24 hours after infection

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4) Can occur in both adults and children 5) Transmission via salivary droplets 6) The presence of N. meningitis within the nervous system causes a massive response by neutrophils resulting in inflammation, which obstructs the normal flow of fluids causing infarcts

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7) The bacteria and leukocytes metabolize the glucose normally found in cerebrospinal fluid potentially depriving the brain of nutrients 8) In addition, N meningitis circulates in blood and produces an endotoxin that causes a drop in blood pressure leading to shock

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B) Streptococcus pneumonia 1) Referred to as the pneumococcus 2) Most frequent cause of community-acquired meningitis 3) Very severe

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C) Haemophilus influenza 1) Tiny gram-negative pleomorphic rods 2) Causes severe meningitis

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D) Listeria monocytogenes 1) Gram-positive bacillus 2) Foodborne disease a) Commonly contracted from eating Mexican cheese, soft cheeses, non-pasteurized milk, hot dogs, or coleslaw

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3) The bacteria can easily penetrate the intestinal linings and enter the bloodstream, it then crosses over into the cerebrospinal fluids where it can then infect the meninges 4) The organism can grow in commercially prepared foods at refrigerator temperatures and has resulted in thousands of infections originating from a single food-processing plant

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5) In normal adults – mild infection with nonspecific symptoms of fever, diarrhea, and sore throat 6) In elderly or immunocompromised patients, fetuses, or neonates – affects the brain and meninges and results in septicemia

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B. Fungal Meningitis
1. Cryptococcus neoformans
A) Fungal disease which originates as a lung infection after a person inhales the spores
1) Often found in dust laden with pigeon droppings

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B) More chronic form of meningitis 1) Rarely invades the nervous system of healthy people but they can be a threat to the life of individuals with underlying diseases such as diabetes, cancer, and immunodeficiency 2) Headache – most common symptom; also nausea and stiff neck C) Person-to-person transmission does not occur

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C. Viral Meningitis 1. Sometimes called aseptic meningitis because the CSF is free of pathogens 2. Majority of cases occur in children 3. 90% caused by enteroviruses A) Can also be caused by a number of herpes viruses

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4. Much more common than bacterial meningitis, it causes similar but much milder symptoms with recovery in 7 to 10 days 5. The viruses are transmitted via the fecal-oral route, respiratory secretions or saliva

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D. Neonatal Meningitis 1. Almost always a result of infection transmitted by the mother, either in utero or during passage through the birth canal

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2. Two most common causes A) Streptococcus agalactiae – Group B strep 1) Most common cause B) Escherichia coli 3. More common in premature babies with immature immune systems

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E. Acute Encephalitis 1. Encephalitis can present as acute or subacute 2. Always a serious condition

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3. Acute: almost always caused by viral infection A) Signs and symptoms vary but may include behavior changes, confusion, decreased consciousness, and seizures B) Multiple causative agents but all are arborviruses C) Usually spread by mosquitoes

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D) Common symptoms include an acute fever accompanied by a rash; however can cause serious illness resulting in permanent disability or death E) Multiple types 1) Western Equine Encephalitis (WEE) 2) Eastern Equine Encephalitis (EEE)

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3) California Encephalitis 4) St. Louis Encephalitis (SLE) a) May be most common of all American viral encephalitides 5) West Nile Encephalitis

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F) Herpes Simplex Virus 1) Can cause encephalitis in newborns born to HSV-positive mothers

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G) JC Virus 1) Infection is common but rarely causes symptoms in otherwise health patients 2) In patients with immune dysfunction, cause progressive multifocal leukoencephalopathy (PML) – uncommon but generally fatal

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4. Subacute Encephalitis
A) Symptoms take longer to show up and are less striking
B) Most common cause is Toxoplasma gondii
1) Flagellated parasite

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C) Most cases go unnoticed 1) Asymptomatic or marked by mild, nonspecific symptoms such as sore throat, lymph node enlargement, and low-grade fever D) In the fetus and immunodeficient people, the disease is severe and often fatal

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F. Rabies 1. Slow, progressive, disease characterized by fatal meningoencephalitis 2. Caused by the rhabdovirus A) A RNA virus with a distinctive bullet shape 3. The virus is spread to humans from wild and domestic reservoirs via bites, scratches, and sometimes inhalation of respiratory droplets

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4. The virus initially stays at the entry site and multiplies before moving along sensory nerves to the CNS 5. Viral replication in the CNS is followed by migration to structures such as the eye, heart, skin, and salivary glands (which completes the cycle)

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6. The disease progresses through identifiable stages A) Incubation – days 1) Occurs at the wound site 2) Some feel pain, burning, & tingling

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B) Prodromal Stage – 2-10 days 1) Characterized by fever, anorexia, nausea, vomiting, headache, and fatigue

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C) Neurological phase – 2-7 days; may present in one of two forms 1) Furious form a) Agitation, disorientation, seizures, and twitching b) Hydrophobia also is seen because of the pain involved with swallowing 2) Dumb form a) Patient us usually paralyzed & disoriented

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D) Coma – 0-14 days E) Death

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7. Diagnosis is difficult because symptoms of rabies often mimic other diseases A) Often occurs postmortem 8. There is no known effective treatment once symptoms develop A) Pre-symptom individuals are given rabies immune globin immediately and a series of 5 vaccinations over 28 days

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G. Poliomyelitis (Polio) 1. Acute enteroviral infection of the spinal cord 2. Is currently the focus of an international campaign to rid the Earth of the disease 3. Caused by polioviruses 1, 2, and 3 A) All are members of the picornavirus family

38. Diseases of the Nervous System
4. Poliovirus enters the body orally (fecal-oral), infects the throat and intestinal tract, invades the bloodstream, and then crosses the blood-brain barrier 5. The virus selectively destroys motor nerve cells of the brain and spinal cord leading to paralysis, muscle wasting, failure of normal bone development and death

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6. May or may not cause paralysis depending on severity of disease A) Nonparalytic – invasion but no destruction of nervous tissue B) Paralytic – various degrees of flaccid paralysis

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7. Post-polio syndrome occurs years (15-50 years later) after acute poliomyelitis infection A) Affects about 25-50% of patients B) Probably caused by the death of nerve cells that had taken over for the one’s killed initially 1) Symptoms include fatigue, slowly progressive muscle weakness, muscular atrophy, joint pain and scoliosis

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8. Prevention includes the use Salk’s inactivated, injectable vaccine in most areas or Sabin’s orally administered attenuated vaccine in areas of epidemic or endemic diseases

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H. Tetanus 1. Caused by Clostridium tetani A) Gram-positive, spore-forming rod B) Releases a powerful neurotoxin, tetanospasmin, that binds to target sites on peripheral motor neurons, spinal cord and brain, and in the sympathetic nervous system

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1) Toxin blocks the inhibition of muscle contraction resulting in involuntary skeletal muscle contractions 2) Contractions are intermittent but can be very painful; jaw muscles are the first affected = lockjaw 3) Death can occur if respiratory muscles are affected

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C) Treatment includes antibiotics for the infection and administration of Tetanus Immune Globulin (TIG; an antitoxin)

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I. Botulism 1. Although botulism is not a nervous system infection, it is a common type of food poisoning that can cause paralysis and be fatal 2. Caused by Clostridium botulinum A) An anaerobic, spore-forming, Gram-positive rod

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3. Three major forms A) Food-borne botulism – occurs in adults & children 1) Ingestion of preformed toxin

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B) Infant botulism 1) Ingestion of bacteria or spores is followed by an infection releasing botulinum toxin into the bloodstream 2) Some cases have been linked to eating raw honey

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C) Wound botulism 1) The bacteria or spores colonize a dirty wound especially those containing dead tissue and release botulinum toxin into the bloodstream

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4. Symptoms result from the release of powerful neurotoxins which work by blocking the release of neurotransmitters A) Initial symptoms include double vision, difficulty in swallowing, and dizziness B) Later symptoms include descending muscular paralysis and respiratory compromise which can cause death

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J. African Sleeping Sickness (trypanosomiasis) 1. Caused by Trypanosoma brucei 2. The causative agent is transmitted to humans via the bite of a tsetse fly 3. There are two common subspecies

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A) T. brucei rhodesiense infection is more severe with heart and brain invasion within 6 weeks of infection and death within 6 months B) T. brucei gambiense infection progresses more slowly and years may pass before death occurs, often from secondary infection

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4. Initially the organism multiplies in the skin at the site of infection but within a few weeks it enters the lymphatics and blood circulation 5. Usually a long asymptomatic period precedes onset of symptoms 6. Once the nervous system is involved the patient experiences uncontrollable sleepiness, headache, poor concentration, unsteadiness, coma, and death