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Case Management. JC. 39/ F From Ermita , Manila DOA: December 8 2009 Married Right handed Roman Catholic Non-hypertensive, non-diabetic, non-asthmatic Chief complaint: Difficulty of breathing. JC’s Profile.
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JC • 39/ F • From Ermita, Manila • DOA: December 8 2009 • Married • Right handed • Roman Catholic • Non-hypertensive, non-diabetic, non-asthmatic • Chief complaint: Difficulty of breathing
JC’s Profile • Known case of Multiple Sclerosis (MS) in 2006 (PGH), supported by MRI and CSF studies • Had 3 admissions for MS relapse • Previous admissions: • July 2006 • November 2008 • November 2009 • Maintenance medication: • Polyneuro 1000 mg/tab OD • Gabapentin 400 mg/cap at HS
History of Present Illness 3 days PTA • Patient noted sudden onset difficulty of breathing slightly relieved by O2 supplementation • (+) light-headedness, nonvertiguous dizziness, and anorexia • No headache, no weakness, no numbness, no vomiting, no chest pain, no blurring of vision, no slurring of speech
History of Present Illness 2 days PTA • Persistence of previously mentioned symptoms • Inability to move right upper extremity up to the right hand and fingers • Dysphonia • Dysarthria • No numbness, paresthesia, shooting pain • Consult at PGH and admission
Review of Systems • (-) headache, seizure, loss of consciousness • (-) fever, cough, colds • (-) vomiting, abdominal pain • (-) orthopnea, palpitations, PND • (-) dysuria, bowel changes • (-) petechiae, ecchymoses, edema, cyanosis, erythema, pruritus
Past Medical History • (-) Hypertension • (-) Diabetes • (-) Bronchial Asthma • (-) PTB • 3 previous admissions in PGH for Multiple Sclerosis relapse • s/p Tracheostomy in 12/2008 for Acute respiratory failure
Family History • No known case of Multiple Sclerosis • No family member presenting with similar symptoms • (-) Hypertension • (-) Diabetes • (-) BA • (-) PTB • (+) stroke - uncles
Personal and Social History • Married and unemployed • Graduate of Commerce • Lives with husband and younger sister in Ermita Manila • Non-smoker • Non-alcoholic beverage drinker • Bed bound and wheelchair borne • Needs assistance on ADLs (self-care, transfer, bathing) • Able to feed herself
Menstrual and Obstetric History • Menarche: 12 y/o • G 1 P 1 (1001), SVD • Menstruation : RMI interval, 3-5 duration, 3 pads per day, (+) dysmenorrhea
Physical Examination • Awake, coherent, conversant • With tracheostomytube, tracheostomy tube in place • MV setting: RR 16, PEEP 60, FiO2 40% • Can tolerate brief periods of room air, but still unable to tolerate weaning from MV • Dysphonic • Not in respiratory distress • Spontaneous eye opening, oriented, follows commands
Physical Examination • BP 110-160 / 90-100 • HR 92-100 • RR 24 • T 38 – 39.5 ‘ C (since 5 days ago)
Neurologic Examination • Awake, conscious, coherent, conversant but dysphonic • GCS 15: Spontaneous eye movement, oriented to time/place/person, follows commands • Good recent and remote memory • Scored 30/30 in MMSE • (-) dyscalculia, (-) Right to Left confusion
Neurologic Examination Cranial 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 intact • V - good corneal reflex, intact sensory V1, V2, V3 • VII - no facial asymmetry • VIII - Good gross hearing • IX, X - Good gutturals, fair gag, midline uvula • XI – Weak shoulder shrug R>L • XII - Tongue midline
3++ 3+ 3+ 3++ Neurologic Examination • MMT • Sensory exam • Generalized atrophy, more extensive at Bilateral lower extremities • Hypertonic Right LEx, hypotonic Right UEx • ( - ) Babinski, ( - ) clonus • ( + ) abdominal reflex
Neurologic Examination • (-) nystagmus • (-) dysmetria • (-) dysdiadochokinesia • (-) Kernig sign • (-) Brudzinski • (-) nuchal rigidity • (-) abnormal sweat pattern
Past Working Impression Multiple Sclerosis in relapse Acute respiratory Failure 2’ MS • s/p MPPT (12/2009) • s/p Tracheostomy (12/2009)
12/8/09 Admitted Course in the Ward 12/25/09 MPPT 12/12/09 BT 1 pack pRBC 01/08/10 Unable to wean ETA CS: P. aeruginosa 02/01/10 Febrile 38-39 Ertapenem Ciprofloxacin January 12/10/09 Febrile rhonchi December • February 1/2/10 MRI: 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 12/11/09 Self-extubated and reintubated 02/11/10 Given Doripenem 12/9/09 Intubated Referral to Pulmo and Rehab 02/08/10 U/A: WBC : 30-35 Urine GS: NG2D Trach CS: NG2D Blood CS : NG2D 12/13/09S s/p Tracheostomy
Current Working Impression • Multiple Sclerosis, in relapse • Optic neuritis • t/c HAP, VAP, UTI • s/p MPPT (12/2009) • s/p Tracheostomy (12/2009)
Multiple Sclerosis • Autoimmune disease • Inflammatory response on myelin sheath • Triad: Inflammation, demyelination and gliosis • Course • relapsing-remitting • Progressive • Initially remitting then becomes progressive
ETIOLOGY • Cause is still unknown • Identified factors: • Autoimmune causes • Human Leukocyte Antigens • Viral causes • Roseola virus
PATHOPHYSIOLOGY Viral infection and autoimmune CNS reaction resulting to abnormal expression of autoantigens (MBP) T-cell sensitization to myelin Demyelination Macrophage infiltration and adhesion to endothelial cells Astrocytes increase in number and size Think, matted fibroglial tissue Wallerian degeneration of axons Partial remyelination or cavitation
Pathogenesis • Etiology • Neural antigens are processed by antigen presenting cells in lymph nodes and presented to T cells • Sensitized memory T cells migrate to the CNS, where they are reactivated by antigen presenting macrophages • Proinflammatory 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 recruitment • Inflammatory response leads to localized demyelination
PATHOPHYSIOLOGY • Scattered areas of demyelination= • “Plaques” • Plaques are more common in: • Optic tracts • Spinal cord • Brain stem • Basal Ganglia • Neurologic Deficits of our patient: • VA at Counting finger, OU • MMT 4/5 for bilateral upper extremities • MMT 0/5 for bilateral lower extremities • Sensory loss 70% in upper extremities • Sensory loss 100% in lower extremities • Dyspnea and repeated respiratory failure • No tremors, chorea
Neurologic Emergency • Multiple 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.
Presentations Acute Transverse Myelitis Optic Neuritis Acute Disseminated Encephalitis
Acute Fulminant Multiple Sclerosis a variant that presents rapidly and may progress to extensive morbidity or mortality in a few days
Neurologic Emergency The 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 Presentations Multiple Sclerosis Sensory Loss Motor (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 Depression 5-10% of patients develop an overt psychiatric disorder (eg, manic depression, paranoia, major depression) or dementia Eye symptoms (diplopia) Trigeminal neuralgia
Clinical Presentations • Optic Neuritis • Demyelination of the optic nerve • 50% of patients with ON have MS • Unilateral blurring of vision, decreased acuity and color perception, discomfort • Phosphenes, Uhthoff phenomenon, Pulfrich effect
Clinical Presentations • Acute Transverse Myelitis • Partial acute transverse myelitis (rather than total) is usually indicative of MS • Devic Syndrome • Acute transverse myelitis accompanied by optic neuritis
Clinical Presentations • Acute Disseminated Encephalitis • Pathophysiologically and radiologically identical to MS • Acute 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 Unusual • patients with MS often present with the other MS presentations such as • Aphasia or dysphasia • Hemianopsia • Seizures (5% of patients with MS) • Significant motor complaints without sensory deficits or dysautonomia (eg, bladder)
Physical Examination • Eye Findings • Optic Neuritis • Impaired visual acuity • Optic atrophy • Papillitis • Afferent papillary defect in affected eye • Incomplete or slow eye movements • Nystagmus • Abnormal papillary responses
Physical Examination • Spinal Cord Involvement • Acute transverse myelitis • Sphincter paralysis and unchanging level • Paralysis, spasticity and hyperreflexia • Decreased joint position and vibration sense • Decreased pain and temperature • Bladder, bowel and sexual dysautonomias
Physical Examination • Cerebellar Findings • Disequilibrium • Truncal or limb ataxia • Scanning (ie, monotonous) speech • Intention tremor • Saccadic dysmetria • Lhermitte sign • Neck flexion results in an electric shocklike feeling in the torso or extremities
Physical Examination • Acute disseminated encephalitis • Altered mental status and/or personality changes • Cranial nerve defects • Hemiparesis • Focal seizures • Autonomic dysfunction • Cranial nerve defects • Ataxia • Dysphasia • Meningismus –symptoms of meningeal irritation without actual inflammation
Treatment Goals • Management of acute excaerbation • Modify disease progression • Symtomatic control • MS IS NOT CURABLE!
Management of acute exacerbations • Glucocorticoids: reduce severity and shortens duration of attacks, suppress PML migration • IV methylprednisolone 500-1000mg/d 3-5 days ± oral prednisone 60-80 mgd • Plasmapheresis (7 exchanges: 54ml/kg every other day for 14 days • costly
Mangement of acute exacerbations • Identify and control precipitants • Treat infections • Normalize body temperature
Disease modifying therapies • Immune-modulators • IFN-β, Glatiramer • Immunosuppresants • Mitxantrone
IMMUNE-MODULATORS • INTEFERON β • Downregulatingexpession of MHC on antigen presenting cell • Inhibiting proinflammatory and increasing regulatory cytokine levels • Inhibition of T cell proliferation • Limiting traffic of inflammatory cells to the CNS • Reduces attack rate & improves disease severity measures (EDSS progression and MRI-doc disease burdern
IMMUNE-MDULATOR • GLATIRAMER acetate (random mixtire of polypeptide) • Induction of antigen-specifis suppressor T cell • Binding MHC molecules • Altering balance between proinflammatory • & regulatory cytokines • reduces attack rate (essp in RR MS)