Case Management

Case Management

Case ManagementJC39/ FFrom Ermita, ManilaDOA: December 8 2009MarriedRight handedRoman CatholicNon-hypertensive, non-diabetic, non-asthmatic

Chief complaint: Difficulty of breathingJCs ProfileKnown case of Multiple Sclerosis (MS) in 2006 (PGH), supported by MRI and CSF studiesHad 3 admissions for MS relapsePrevious admissions: July 2006November 2008November 2009 Maintenance medication:Polyneuro 1000 mg/tab ODGabapentin 400 mg/cap at HSHistory of Present IllnessPatient noted sudden onset difficulty of breathing slightly relieved by O2 supplementation(+) light-headedness, nonvertiguous dizziness, and anorexiaNo headache, no weakness, no numbness, no vomiting, no chest pain, no blurring of vision, no slurring of speech3 days PTAHistory of Present IllnessPersistence of previously mentioned symptomsInability to move right upper extremity up to the right hand and fingersDysphoniaDysarthriaNo numbness, paresthesia, shooting pain

Consult at PGH and admission2days PTAReview of Systems(-) headache, seizure, loss of consciousness(-) fever, cough, colds(-) vomiting, abdominal pain(-) orthopnea, palpitations, PND(-) dysuria, bowel changes(-) petechiae, ecchymoses, edema, cyanosis, erythema, pruritusPast Medical History(-) Hypertension(-) Diabetes(-) Bronchial Asthma(-) PTB3 previous admissions in PGH for Multiple Sclerosis relapses/p Tracheostomy in 12/2008 for Acute respiratory failure

Family HistoryNo known case of Multiple SclerosisNo family member presenting with similar symptoms(-) Hypertension(-) Diabetes(-) BA(-) PTB(+) stroke - unclesPersonal and Social HistoryMarried and unemployedGraduate of CommerceLives with husband and younger sister in Ermita ManilaNon-smokerNon-alcoholic beverage drinkerBed bound and wheelchair borneNeeds assistance on ADLs (self-care, transfer, bathing)Able to feed herself

Menstrual and Obstetric HistoryMenarche: 12 y/oG 1 P 1 (1001), SVDMenstruation : RMI interval, 3-5 duration, 3 pads per day, (+) dysmenorrheaPhysical ExaminationAwake, coherent, conversantWith tracheostomy tube, tracheostomy tube in placeMV setting: RR 16, PEEP 60, FiO2 40%Can tolerate brief periods of room air, but still unable to tolerate weaning from MVDysphonicNot in respiratory distressSpontaneous eye opening, oriented, follows commandsPhysical ExaminationBP 110-160 / 90-100HR 92-100RR 24T 38 39.5 C (since 5 days ago)

Physical ExaminationSystemFindingsHEENTPink palpebral conjunctivae, anicteric sclerae, (-) neck vein engorgement, (-) cervical lymphadenopathy, (-) suprqaclavicular retractionsChestSymmetrical chest expansion, clear breath sounds, (-) wheezes, (-) crackles, (+) rhonchiCardiacAdynamic precordium, distinct heart sounds, normal rate and regular rhythm, no murmurs appreciatedAbdomenFlat and soft, normoactive bowel sounds, nontender on light and deep palpation, liver edge not palpable, intact Traubes spaceExtremitiesPink nailbeds, no cyanosis, no edema, full and equal pulses, CRT < 2 sec, (+) atrophy of bilateral thenar area and bilateral quadriceps and gastrocnemiusNeurologic ExaminationAwake, conscious, coherent, conversant but dysphonicGCS 15: Spontaneous eye movement, oriented to time/place/person, follows commandsGood recent and remote memoryScored 30/30 in MMSE(-) dyscalculia, (-) Right to Left confusionNeurologic ExaminationCranial Nerves:II -VA finger counting OU; 3 mm EBRTL D & C; (+) RAPD; Funduscopy: (+) ROR, clear media, (-) papilledema (-) disc atrophy, III, IV, VI - 1 gaze midline, EOMS full and intactV - good corneal reflex, intact sensory V1, V2, V3VII - no facial asymmetryVIII - Good gross hearingIX, X - Good gutturals, fair gag, midline uvulaXI Weak shoulder shrug R>LXII - Tongue midlineNeurologic ExaminationMMT

Sensory exam

Generalized atrophy, more extensive at Bilateral lower extremitiesHypertonic Right LEx, hypotonic Right UEx( - ) Babinski, ( - ) clonus( + ) abdominal reflex

4/54/50/50/530% sensory loss at T130% sensory loss at T10 % 3+3++3++

3+Neurologic Examination(-) nystagmus(-) dysmetria(-) dysdiadochokinesia(-) Kernig sign(-) Brudzinski(-) nuchal rigidity(-) abnormal sweat patternPast Working ImpressionMultiple Sclerosis in relapseAcute respiratory Failure 2 MSs/p MPPT (12/2009)s/p Tracheostomy (12/2009)

DecemberCourse in the WardJanuaryFebruary12/8/09Admitted12/9/09IntubatedReferral to Pulmo and Rehab12/10/09Febrilerhonchi12/11/09Self-extubated and reintubated12/13/09Ss/p Tracheostomy12/25/09MPPT1/2/10MRI: Patchy areas of intrinsic signal abnormalities within the cervico-medullary junction and cervical cord (@ C3-4 level), associated w/ patchy contrast enhancement consistent w/ M.S. No associated cord expansion. No evidence of cord compression

01/08/10Unable to weanETA CS: P. aeruginosa02/01/10Febrile 38-39ErtapenemCiprofloxacin02/08/10U/A: WBC : 30-35Urine GS: NG2DTrach CS: NG2DBlood CS : NG2D12/12/09BT 1 pack pRBC02/11/10Given Doripenem12/10/2009Febrile episode, rhonchiA> r/o CAP vs Pulmonary EmboliP> Diagnostics done: ABG< U/A, ETA GS/CSCeftazidime 1 gm IV q8h ANSTClindamycin 300 mg cap q6hParacetamol 500 mg tab q4 PRN for T>37.5CSuction secretion PRN

19Current Working ImpressionMultiple Sclerosis, in relapseOptic neuritist/c HAP, VAP, UTI

s/p MPPT (12/2009)s/p Tracheostomy (12/2009)DiscussionMultiple SclerosisAutoimmune disease Inflammatory response on myelin sheathTriad: Inflammation, demyelination and gliosisCourse relapsing-remittingProgressiveInitially remitting then becomes progressiveETIOLOGYCause is still unknownIdentified factors:Autoimmune causesHuman Leukocyte AntigensViral causesRoseola virus

PATHOPHYSIOLOGYDemyelinationMacrophage infiltration and adhesion to endothelial cellsAstrocytes increase in number and sizeThink, matted fibroglial tissueWallerian degeneration of axonsPartial remyelination or cavitationViral infection and autoimmune CNS reaction resulting to abnormal expression of autoantigens (MBP) T-cell sensitization to myelinPathogenesisEtiologyNeural antigens are processed by antigen presenting cells in lymph nodes and presented to T cellsSensitized memory T cells migrate to the CNS, where they are reactivated by antigen presenting macrophagesProinflammatory cytokines are secreted. Enhance expression of adhesion molecules by vascular endothelium, alter permeability of the blood-brain barrier, and induce a second wave of inflammatory cell recruitmentInflammatory response leads to localized demyelinationPATHOPHYSIOLOGYScattered areas of demyelination= PlaquesPlaques are more common in:Optic tractsSpinal cordBrain stemBasal GangliaNeurologic Deficits of our patient:

VA at Counting finger, OU MMT 4/5 for bilateral upper extremitiesMMT 0/5 for bilateral lower extremitiesSensory loss 70% in upper extremitiesSensory loss 100% in lower extremities Dyspnea and repeated respiratory failureNo tremors, choreaNeurologic EmergencyMultiple Sclerosis typically progresses over decades presenting with mild symptoms and some exacerbations

In some cases, however, it can progress so rapidly as to lead to major exacerbations and even sudden onset of symptoms and death in the course of a few days.

PresentationsAcute Transverse MyelitisOptic NeuritisAcute Disseminated Encephalitis

MS Variants and Severity

Acute Fulminant Multiple Sclerosisa variant that presents rapidly and may progress to extensive morbidity or mortality in a few days

Neurologic EmergencyThe majority of MS patients are sent to the emergency due to relapse of the previously managed condition or an acute exacerbation of the symptoms.Clinical PresentationsMultiple Sclerosis Sensory LossMotor (eg, muscle cramping secondary to spasticity)Autonomic (eg, bladder, bowel, sexual dysfunction)Cerebellar symptoms (eg, Charcot triad of dysarthria, ataxia, tremor)Constitutional symptoms (fatigue and dizziness)Subjective difficulties with attention span, concentration, memory, and judgment Depression5-10% of patients develop an overt psychiatric disorder (eg, manic depression, paranoia, major depression) or dementiaEye symptoms (diplopia)Trigeminal neuralgiaClinical PresentationsOptic NeuritisDemyelination of the optic nerve50% of patients with ON have MSUnilateral blurring of vision, decreased acuity and color perception, discomfortPhosphenes, Uhthoff phenomenon, Pulfrich effectClinical PresentationsAcute Transverse MyelitisPartial acute transverse myelitis (rather than total) is usually indicative of MSDevic SyndromeAcute transverse myelitis accompanied by optic neuritis

Clinical PresentationsAcute Disseminated EncephalitisPathophysiologically and radiologically identical to MSAcute onset of motor, sensory, cerebellar, and cranial nerve dysfunction with encephalopathy, progressing to coma and eventual death in 30% of such cases

Multiple Sclerosis as a Sole Symptom is Unusualpatients with MS often present with the other MS presentations such asAphasia or dysphasiaHemianopsiaSeizures (5% of patients with MS)Significant motor complaints without sensory deficits or dysautonomia (eg, bladder)Physical ExaminationEye FindingsOptic NeuritisImpaired visual acuityOptic atrophyPapillitisAfferent papillary defect in affected eyeIncomplete or slow eye movementsNystagmusAbnormal papillary responses

Physical ExaminationSpinal Cord InvolvementAcute transverse myelitisSphincter paralysis and unchanging levelParalysis, spasticity and hyperreflexiaDecreased joint position and vibration senseDecreased pain and temperatureBladder, bowel and sexual dysautonomiasPhysical ExaminationCerebellar FindingsDisequilibriumTruncal or limb ataxiaScanning (ie, monotonous) speechIntention tremorSaccadic dysmetria

Lhermitte signNeck flexion results in an electric shocklike feeling in the torso or extremities

Physical ExaminationAcute disseminated encephalitisAltered mental status and/or personality changesCranial nerve defectsHemiparesis Focal seizuresAutonomic dysfunctionCranial nerve defectsAtaxiaDysphasiaMeningismus symptoms of meningeal irritation without actual inflammationTreatment GoalsManagement of acute excaerbationModify disease progressionSymtomatic control

MS IS NOT CURABLE!

Management of acute exacerbationsGlucocorticoids: reduce severity and shortens duration of attacks, suppress PML migrationIV methylprednisolone 500-1000mg/d 3-5 days oral prednisone 60-80 mgdPlasmapheresis (7 exchanges: 54ml/kg every other day for 14 dayscostlyMangement of acute exacerbationsIdentify and control precipitantsTreat infectionsNormalize body temperatureDisease modifying therapiesImmune-modulatorsIFN-, GlatiramerImmunosuppresantsMitxantroneIMMUNE-MODULATORSINTEFERON Downregulating expession of MHC on antigen presenting cellInhibiting proinflammatory and increasing regulatory cytokine levelsInhibition of T cell proliferationLimiting traffic of inflammatory cells to the CNSReduces attack rate & improves disease severity measures (EDSS progression and MRI-doc disease burdern

Exanded disability Status score49IMMUNE-MDULATORGLATIRAMER acetate (random mixtire of polypeptide)Induction of antigen-specifis suppressor T cellBinding MHC moleculesAltering balance between proinflammatory& regulatory cytokines reduces attack rate (essp in RR MS)Glu tyr ala lysine50IMMUNOSUPPRESANTSMITOXANTRONE (anthracendione)Interacalates DNAInterfere with RNA synthesisfirst drug approved for the treatment of secondary progressive multiple sclerosis (SPMS)Beneficial effect on disease progression in patients with MS whose clinical condition is deteriorating (Type B recommendationlimited use and of potentially great toxicityReserved for patients with rapidly advancing disease who have failed other therapies

Symptomatic TherapySpasticity: may improve with physical therapy, regular exercise & stretchingRx: Lioresal (20-120mg/d), diazepam 2-40 mg/d, dantroline (25-40mg/d)Pain: Rx: carbamazepine (100-1000mg/d), phenytoin (300-600mg/d) gabapentin

Symptomatic TherapyAtaxia/tremor: clonazepam (1/5-20 mg/d), propranolol (40-200mg/d)Bladder Dysfunction:Evening fluid restriction and frequent voluntary voidingIntermittent catherization for UTI preventionConstipation: high fiber diet, laxatives may be use

Rehabilitation and Social SupportOccupational therapy: handling ADLSPhysical therapy: mobility techniquesPsychological support: for coping and management of depressionManagement in our PatientPrevious managements/p MPPT: Methylprednisolone 500 mg vial dissolve in 250 cc D5W to run in 6 hours OD x 3 daysGabapentin 400 mg cap at HSPolynerv 1000 mg tab ODHeparin 5000 u SQ BIDCeftazidime 1 gm IV q8h ANSTClindamycin 300 mg cap q6hParacetamol 500 mg tab q4 PRN for T>37.5CDolcet 1 tab q8hDulcolax 1 tab at HSNaHCO3 50 meqsSalbutamol 1 neb q8h alternate with pNSS 3cc q 8hOmeprazole 40 mg cap OD via NGTShifted Ceftazidime to Piper-Tazo 2.25 gm IV q8h ANSTItopride 10 mg tab TIDCiprofloxacin 750 mg tab BIDCelecoxib 200 mg cap BID Salbutamol neb q8 PRN Citalopram 20mg tab ODBaclofen 10 mg tab TID Myonal 50 mg tab BIDN-Acetylcysteine 600 mg in 50 cc H2O BIDDoxofylline 100 mg/5 ml 10 ml ODCurrent managementParacetamol 500 mg Q4 Oxycontin 10mg/capErtapenem 1 g IV OD Salbutamol neb Q8Ciprofloxacin 500 mg/tab Dulcolax 1 tab ODShifted to Doripenem 500 mg IV OD

PROGNOSISFAVORABLE FACTORS:Females Low rate of relapses per year Complete recovery from the first attack Long interval between first and second attack Symptoms predominantly from afferent systems (i.e. sensory symptoms) Younger age of onset PROGNOSISFAVORABLE FACTORS:Low disability at 2 to 5 years from the disease onset Later cerebellar involvement Involvement of only one CNS system at the time of onset PROGNOSISUNFAVORABLE FACTORS:Males High rate of relapses per year Incomplete recovery from the first attack Short interval between first and second attack Symptoms predominantly from efferent systems (i.e. symptoms of motor tract involvement) Older age of onset PROGNOSISUNFAVORABLE FACTORS:Significant disability at 2 to 5 years from the onset acute onset Early cerebellar involvement Involvement of more than one CNS system at the time of onset PROGNOSISAverage life span after diagnosis is 25 to 35 yearsSuicide rate is 7.5 times higher Common causes of deathCompromised swallowing and breathingSevere infections (e.g. Urosepsis, Aspiration pneumonia)

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