Inflammations of the nervous system

Inflammations of the nervous systemDynamics•acute•monophasic•biphasic•subacute•fluctuating•chronic•intermittent•progressiveLocalization•meningitis•encephalitis•abscess•epidural empyema•sinusthrombophlebitis

Pathology1. Meningeal infiltration2. Blood-brain barrier damage• perivascular cellular infiltration• extracellular edema3. Glial proliferation4. Damage of the nervous tissue(neuron, axon, oligodendroglia, myelin sheath)The routes of infection1. Hematogen (abscess)2. Paranasal sinuses (meningitis)3. Respiratory tract (nose, lung) (meningoencephalitis)4. Scull fracture (abscess, epiduralempyema, meningitis)5. Peripheral nerve (rabies, HSV, VZV)

MENINGITISPathology•Cellular infiltration of the meningxes•Brain parenchyma is intact, except edemaand radicular/cranial nerve lesionsClinical picture: ("Meningeal syndrome”)• general symptoms (fever, weakness)• signs of meningeal irritation (nuchalrigidity, Kernig-, Brudzinsky signs,headache, hyperirritability)• changes in mental status (apathy,somnolence, delirium)• focal neurological signs are absent• "CSF syndrome" (increased cell&protein)

Acute aseptic meningitisPathogens:•virusHERPES (HSV, HZV, EBV, CMV),ENTERO (echo, coxaci, polio, etc.),ARBO (tick-bite encephalitis virus),ADENO, LCMV, HIV, etc.•spirochete (leptospira, borrelia, treponema)Clinical pictureIncubation 7-10 day, "dromedary" courseCSF:•cell count 50-1000/ul, lymphocytes•protein level moderately elevated (0.4-lg/1)•sugar: normal•microbiol. culture: negative Dg: serology(repeated)Others:EEG, neuroimaging: usually normalTherapy: supportive

Subacute/chronic meningitisPathogens:•mycobacterium, mycoplasma•fungus (candida, cryptococcus, aspergillus)•spirochete (leptospira, borrelia)•virus (LCMV)•toxoplasma•non-infective (leukemia, SLE, tumour cells)Clinical picture (weeks-months)intermittent febrile statesslowly progressive mental changes, mild (orabsent) meningeal signs, progressive cranialnerve palsiesCSF:•50-100/ul cells, lymphocytes•high protein level - 1-3 g/1 (severe BBBdamage)•low sugar level (>2 mmol/l)Dg: need special culture technique, serologyTherapy: for tbc: Isonicid, Rifampycin,Dexamethason. for fungus: Amphotericin B

Purulent meningitisPathogens:•adult: Pnemococcus, N. meningitidis,•child: Hemophylus, EnterobacteriaClinical picture:primary extracranial manifestation (lung,paranasal)severe condition, high fever, prominentmeningeal signs, confusion (high mortality !)residual signs (hydrocephalus, hypacusis,ment. retard., epileptic seizures)CSF: green-like•1000 to several 10,000 cells/ul, granulocytes•high protein level (1-3 g/1)•low sugarDg: microbiol. cultureOther: eradication of the (respiratory) sourceEEG: generalized slowing CT/MRI: look for"pus-cap”, brain abscess, hydrocephalusTherapy: based on the culture. Before:Penicillin + Ampicillin + Ceftriaxon iv., insevere cases + Dexamethason(Steptokinaze)

„Covered syndrome”(pretreated/masked form of purulentmeningitis)Clinical pictureheadache, intermittent febrile state, butno prominent meningeal signsantibiotic therapy in the recent historyCSF•no more than 1000 cells, granulocytes/mixed•protein elevated >1 g/l•sugar: decreased•negative culture resultDiagnosis based on CSF findingsTherapy should be the same as in acutepurulent meningitis (combined antibiotics)Brain abscessEtiol.: direct or haematogenic spreadingClinical picture: focal signs, seizures, risedintracranial pressureDg: CT, MRITH: antibiotics, surgical

ENCEPHALITISPathology:Blood-brain barrier damage+Central nervous system damageClinically: "Encephalitis syndrome"meningeal syndrome +neurological signs (focal signs of motor-,sensory, autonomic systems, sings ofinreased intracranial pressure)seizuresaltered mental stateEEG, MRI, CSF alterationsPolio- leuko- panencephalitis

Polioencephalitis:Herpes simplex encephalitis•Clinical: primary [subclinical] infection withHSV-1, endogenous reactivation,• flu-like phase followed by encephalitislocated to temporal lobe (aphasia, paresis,seizures, psychoorganic syndrome).•Dg: CSF, EEG, MRI, biopsy (PCR),serology.•Treatment with Acyclovir 30mg/bwkg a dayfor weeksTick-born encephalitis•Clinical: tick bite,•flu-like illness followed by meningitis orencephalitis syndrome, predilection forcervical spinal cord motoneurons andtemporal lobe•Dg: typical CSF findings, serology•TH: supportive

HSV

Acute anterior poliomyelitisClinical: gastrointestinal infection, viraemiafollowed by asymmetric flaccid paresis,predilection for large motoneurons in spinalcord and brainstem,Dg: typical CSF findings,Treatment : supportive, prevention withperoral immunization)Lyssa, rabiesClinical: transmitted by bite of an rabidanimal {small predators}, week to months ofincubation period,Stages: 1. Prodrome (fever, fatigue) 2.Excitation (restlessness, hyperirritability,convulsions, 3. Paralytic phase (widespreadparesis, death)Dg. Pathology (Negri-body in hippocampus),CSF, preventive immunization)

CMVNegri

LeukoencephalitisPathology: immune demyelinationClinical picture: acute/subacute, monophasicdisease with widespread white matter lesion,typical CSF (BBB damage +OB)•parainfectious encephalomyelitis(measles, rubella, varicella, smallpox, mumps,infectious mononucleosis)•postvaccination encephalomyelitis(vaccination against smallpox, measles,rabies, tetanus)

Panencephalitis(„slow virus” diseases, progressive course)•SSPE (subacute sclerotising panencephalitis)•CJD (Kreutzfeld-Jakob disease)•PML (progressive multifocalleuko-encephalopathy)

SSPEPathogenesis:childhood infection with measles virusreactivation of "defective" virus (5-10y),Clinical pictureno general signs, young patients,progressive dementia (impaired cognitiveperformance in school), ataxia, myoclonicjerks, decerebration, deathEEG: generalized slow waves(Rademecker complex) in every 3-10 sec.CSF: OB, high anti-measles Ab'sTh: symptomatic

CJDEtiology:Prion (sporadic, iatrogenic, familial)Clinical pictureOnset: 35-65 y, personality changes,progressive dementia within few months,extrapyramidal signs, ataxia, dysarthria,myoclonus, death within a yearsEEG: triphasic slow complexes, 1-2 cpsCSF: unremarcableDg: clinical picture, (brain biopsy)Th: symptomatic

GSS(Gertsmann-Straussler-Scheinkerdisease)AD inheritanceOnset: 40-50 y, duration 1-10 yClin.: spinocerebellar degen. + dementiaFFI(Fatal familial insomnia)Degen. of the medial nucleus ofthalamusClin: lack of sleep + vegetativedisturbancesDg: clinical + EEG/polysomnography

PML(progressive multifocal leukoencephalopathy)Immunsuppression (HIV, cytostatics,leukemia, malignancies)Mechanism: JC papova virus causesoligodendroglia degenerationClinical picture: subacute onset, changingin personality, pramidal signs, ataxia,dementia, death within a few monthsCSF: unremarkableMRI: demyelination (confluent, noenhancement)Dg: brain biopsyTh: stop immunsuppression, symptomatic

NeurosyphilisPathogen: treponema pallidumPrimer genital infection followed byhematogenous spreadingForms:1. Luetic meningitis (early)2. Meningovascular syphilis (months)3. Paralysis progressiva (dementia,psychosis, several years)4. Tabes dorsalis (spinal ataxia)Dg: CSF, serology, MRITh: penicillin

NEUROBORRELIOSISEtiologySpirochetal (Borrelia burgdorferi) infection bybite of a vector insect (tick borne)Symptoms and signsRelapsing-remitting course with multipleorgan affection: skin- ECM, heart-carditis,joints- mono-, polyarthritis, and CNS/PNS -neuroborreliosisStages•(acute) flu-Iike symptoms (headache,myalgia, ECM)•(subacute) meningitis, meningoradiculitis,mono-, polyneuritis (+arthritis, andsometimes carditis)•(chronic) meningo-encephalo-myelitis

NEUROBORRELIOSISCharacteristic neurological forms:•Diplegia facialis-most common form ofneuritis caused by BB. (1-3 weeks)•Bannwarth-Sy. (meningo-polyradiculitis,1-3 months)•Encephalomyelitis (ataxia, memorydisturbances, fatigue, progressivepyramidal signs, disturbances of bladderfunctions, neuritis retrobulbaris, peripheralneuritis, tetraparesis, dementia)MRI: focal white matter lesionsCSF: OBDg: Based on clinical picture + serologyTh:Prolonged course with high doses ofantibiotics (Doxycyclin,Penicillin,Rocephin )

NEUROLOGICAL COMPLICATIONS OFAIDS1. DISEASES WITH DIRECT RELATIONSTO HIV-1•Meningitis (acute, or chronic form)•Encephalopatia (= AIDS-dementiacomplex)•Myelopathy•Neuropathy -symmetric polyneuropathy-mononeuritis multiplex•Myopathy (direct or due to treatment)2. SECONDARY COMPLICATIONS•Opportunistic infections (CMV; Toxopl.,Cryptococcus, PML, HSV, TBC)•Neoplasms (lymphoma, metastases, etc)•Cerebrovascular diseases (vasculitis)•Metabolic disorders (Wernicke, vitamindeficyency)

HIV