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Case Report

MULTIPLE SCLEROSIS

By:
Fadel Al Habsy

Supervisor:
dr. Riki Sukiandra, Sp.S

DEPARTMENT OF NEUROLOGY
MEDICAL FACULTY OF RIAU UNIVERSITY
ARIFIN ACHMAD GENERAL HOSPITAL
PEKANBARU
2019
KEMENTRIAN PENDIDIKAN DAN KEBUDAYAAN
FAKULTAS KEDOKTERAN UNIVERSITAS RIAU
SMF/ BAGIAN SARAF
Sekretariat : Gedung Kelas 03, RSUD Arifin Achmad Lantai 04
Jl. Mustika, Telp. 0761-7894000
E-mail : saraffkur@gmail.com
PEKANBARU

I. Patient’s Identity

Name Mr. I
Age 43 years
Gender Male
Address Desa, Gunung Sahilan, Kampar
Religion Islam
Marital’s Status Married
Occupation ex-farmer
Entry Hospital February, 14th 2019
Medical Record 010084xx

II ANAMNESIS :
Auto & Alloanamnesis (February, 25th 2019)
A. Chief Complain
The weakness and numbness on both legs

B. Present illness history


4 months before, the patient has complained weakness on both legs, the weakness appears
simultaneously on the right and left legs, it feels the same on all legs, occurs suddenly, both
of the leg couldn’t be lifted, and he couldn’t walk anymore. Besides, the patient also
complained of high fever throughout the day. Fever is not accompanied by shivering. The
temperature decreased temporary by fever medicine that patient had purchased over the
counter.
Patients also felt the numbness from lower abdomen area to his legs and he has complaint
in controlling his urination and defecation. Then the patient treated in Santa Maria Hospital
doing the MRI. The neurologist there said the patient got a spinal chord infection. Patient
and patient’s wife did not know what treatment given there and then patient taken home after
9 days later. The patient always control of his disease in the hospital and the symtoms
become better day by day.
2 months before, the patient has complained again weakness and numbness on both
legs. He still can walk but need help with cane and sometime he feel that his legs move in
an uncontrollable and unintended way. The patient has disturbance with his memory, he
can’t remember the short event and he also complained that he got the double vision. The
patient treated in Syafira Hospital for 10 days. The neurologist there said the patient
suspected got a spinal cord tumor.
10 days before, the patient back again to the hospital and has complained weakness,
numbness, tingling and pain like burning on both legs. He can walk with cane and he still
feel that his legs move in an uncontrollable and unintended way. Pain felt severe in walking
and decrease with a rest, pain felt like burning sensation, heat and sharp, It occured suddenly
without any previous trauma histories before. Patient also complained of having speech
difficulties but he still understand the conversation. Fever was denied, weight loss denied,
prolonged cough denied. Then patient brought to Syafira Hospital, after that referred to
Arifin Ahmad Hospital to better treatment and MRI examination.

C. Past Illness history


 Cough produced phlegm (-)
 Bump on the back (-)
 Weight loss and appetite (-)
 A history of 6 months medication (-)
 Hypertension (-), Diabetes mellitus (-)
 A history of Cancer (-)
 History of backbone surgery (-)

D. Family Illness History


 A history of TB (-)
 A history of Cancer (-)
RESUME ANAMNESIS
Mr. I, 43 years old admitted to the hospital on February, 14th 2019. The patient has
complained weakness, numbness, tingling and pain on both legs suddenly since 10 day
before. He can walk with cane and he still feel that his legs move in an uncontrollable and
unintended way. Pain felt severe in walking and decrease with a rest, pain felt like burning
sensation, heat and sharp. Patient also felt numbness from her lower abdomen area to his
legs and he has complaint in controlling his urination and defecation. The patient has
problem with his memory, he can’t remember the short event and he also complained that
he got the double vision and speech difficulties. The symtoms start for 4 months ago and
he have received remissions and relapses course problem three times until now.

III. PHYSICAL EXAMINATION

A. Generalized Condition (February, 25th 2019)


Blood Presure : 120/80 mmHg
Heart Rate : 86 bpm
Respiratory : Respiratory rate : 20 x/mnt Type : abdominotorakal
Temperature : 37,3°C
Weight : 55 kg Height : 160 cm
IMT : 21,5 (normal)

B. Neurological status
1) Consciousness : Composmentis GCS : 15 (E4V5 M6)
2) Cognitive Function : Normal
3) Neck Rigidity : Negative

C. Cranial Nerves
1. N. I (Olfactorius )
Right Left Interpretation
Sense of Smell Normal Normal Normal

2. N.II (Opticus)
Right Left Interpretation
Visual Acuity Normal Normal
Normal
Visual Fields Normal Normal
Colour Recognition Normal Normal

3. N.III (Oculomotorius)
Right Left Interpretation
Ptosis (-) (-)
Pupil
Shape Round Round
Side Φ3mm Φ3mm Normal
Extraocular movement Normal Normal
Pupillary reaction to light
Direct + +
Indirect + +

4. N. IV (Trokhlearis)
Right Left Interpretation
Extraocular movement Normal Normal Normal

5. N. V (Trigeminus)
Right Left Interpretation
Motoric Normal Normal
Sensory Normal Normal Normal
Corneal reflex (+) (+)

6. N. VI (Abduscens)
Right Left Interpretation
Extraocular movement Normal Normal
Strabismus (-) (-) Normal
Deviation (-) (-)

7. N. VII (Facialis)
Right Left Interpretation
Tic (-) (-)
Motoric Normal Normal
Normal
Flavour Sense Normal Normal
Tanda chvostek - -

8. N. VIII (Acusticus)
Right Left Interpretation

Hearing sense Normal Normal Normal

9. N. IX (Glossopharyngeus)
Right Left Interpretation
Arkus farings Normal Normal
Flavour sense Normal Normal Normal
Gag Reflex (+) (+)

10. N. X (Vagus)
Right Left Interpretation
Arcus farings Normal Normal
Normal
Dysfonia (-) (-)

11. N. XI (Accesorius)
Right Left Interpretation
Motoric Normal Normal
Normal
Trofi Eutrofi Eutrofi

12. N. XII (Hypoglossus)


Right Left Interpretation
Motoric Normal Normal
Trofi Eutrofi Eutrofi Normal
Tremor (-) (-)
Disartria (-) (-)
D. Motoric
Right Left Interpretation
Upper Extremity
Strength
Distal 5 5
Proximal 5 5 Normal
Tone Normal Normal
Trophy Eutrophy Eutrophy
Involuntary movements (-) (-)
Clonus (-) (-)
Lower Extremity
Strength
Distal 3 3
Proximal 3 3 Paraparese
Tone Spastic Spastic (UMN Type)
Trophy Eutrophy Eutrophy
Involuntary movements (+) (+)
Clonus (-) (-)
Body
Trophy Eutrophy Eutrophy
Involuntary movements (-) (-) Normal
Abdominal Reflex (-) (-)

E. Sensory
Right Left Interpretation
Light Touch
Pain
Temperature
Hypesthesia
Proprioceptive
from T9
 Position
dermatome to
 Two point discrimination
the lower
 Stereognosis
 Graphestesia
 Vibration Not Tested Not Tested

F. Reflex
Right Left Interpretation
Physiologic
Biceps (+) (+)
Physiologic reflex
Triceps (+) (+)
()
Knee (+++) (+++)
Ankle (+) (+)
Pathologic
Babinsky (+) (+)
Chaddock (-) (-)
Hoffman Tromer (-) (-) Pathologic reflex (+)
Openheim (-) (-)
Schaefer (-) (-)
Gordon (-) (-)
Primitive Reflex No Primitive Reflex
Palmomental (-) (-)
Snout (-) (-)

G. Coordination
Right Left Interpretation
Point to point movements Normal Normal
Walk heel to toe Not Tested Not Tested
Difficult to assessed
Gait Not Tested Not Tested
Tandem Not Tested Not Tested
Romberg Not Tested Not Tested

H. Autonom system
Urination : Retensio Urine
Defecation : Couldn’t control
Sweat : Normal

I. Others Examination
a. Laseque : >700
b. Kernig : >1300
c. Patrick : -/-
d. Kontrapatrick : -/-
e. Valsava test : -
f. Brudzinski : - j. Gaze test: downbeat nystagmus (+)
g. Spurling test : -
h. Head Traction : -
i. Valsava test : -

EXAMINATION RESUME
General Status :
 Blood Pressure 120/80 mmHg
 Heart Rate 86 bpm
 Respiratory Rate 20 times per minute
 Temperature 37,3°C
Noble Function : Normal
Neck Stiffness : Negative
Cranial Nerves : Normal
Motoric : Paraparese (UMN Type)
Sensory : Hypesthesia from T9 dermatome to the lower
Coordination : Difficult to assessed
Autonomy : Abnormal urination and defecation
Reflex : Physiologic reflex () Patologic reflex (+)
Others Examination : Nystagmus provokatus (+) downbeat nystagmus

IV. WORKING DIAGNOSIS


CLINICAL DIAGNOSIS : spinal cord lesion/thoracal myelopathy,
brainstem/medulla oblongata lesion, intracerebral
lession
 Paraparese (UMN Type)
 Paresthesia and hypesthesia on T9 dermatome to the lower on the both of leg
 Abnormal urination and defecation
 Dysarthria
 Downbeat nystagmus and diplopia
 Movement disorder
TOPICAL DIAGNOSIS : bihemispheric cortical, 9th thoracal spinal cord
segments and infratentorial
ETIOLOGICAL DIAGNOSIS : suspect multiple sclerosis
SECONDARY DIAGNOSIS : Suspect Acute Transverse Myelitis
DIFFERENTIAL DIAGNOSIS : Spinal cord tumour

V. SUGGESTION EXAMINATION :
 Blood routine
 Blood chemistry
 Lumbal puncture (CSF examination)
 MRI (brain and whole spine)
VI. MANAGEMENT
 Maintanance : IVFD RL 20 dpm
 Anti inflamation : dexamethason 3 x 5 mg per IV
 Gastric Protector : ranitidine 2 x 50 mg per IV
 Neurotropic : Mecobalamin 3 x 500 mg
 Flunarizin 2 x 5 mg
 Betahistine mesylate 3 x 6 mg
 Amitriptyline 0-0-1/2 tab 25mg
VIII. LABORATORY AND RADIOLOGY FINDINGS
1. Blood Routine (July, 12th 2016)
Hb : 15,1 g/dL WBC : 8.490/uL
Ht : 41,3 % PLT : 363.000/uL
2. Blood Chemistry (July, 12th 2016)
- Ureum : 10 mg/dL
- Creatinin : 0,5 mg/dL
- AST : 37 U/L
- ALT : 94* U/L
Interpretation : in normal limit
3. Electrolyte
- Na : 118 mmol/L
- K : 2,43 mmol/L
- Cl : 82,16 mmol/L

4. MRI of brain, cervical and thoracal vertebra with contrast (February, 26th
2019)
Result :
 Medulla oblongata and intramedullare SOL in C7, T1-2, T8-9
 Multiple intracerebral SOL on both of hemispheres
 Et causa suspect multiple sclerosis
DISCUSSION
1. Definition
Multiple sclerosis (MS) is a progressive demyelinating and neuro- degenerative
disease of the central nervous system (CNS) which gradually results in severe
neurological deficits. MS usually presents in early adult life when it has a major impact
on family, and on vocational and social life. Since lack of myelin slows down the
conduction of the action potentials, MS manifests itself as impaired performance
that can have a devastating effect on behavior.1 MS usually involves a more or less

progressive development of neurological symptoms and behavioural deficits. The


exact aetiology and pathogenesis, however, are still unclear despite recent advantages
in understanding this mysterious disease. The disease is characterized by remissions
and relaps, erratic onset and duration symtoms that flare up acutely. 2
2. Epidemiology
3. Etiology and Risk Factors
4. Pathogenesis

Gambar 3. Heterogenitas pada MS. (Dendrou CA,


2015)
Gambar 5. Neurodegeneratif dan inflamasi pada MS. (Dendrou CA, 2015)

Dendrou CA, Fugger L, Friese MA. 2015. Immunopathology of multiple


sclerosis. Nat Rev Immunol. Sep 15;15(9):545-58.

Gambar 5. Neurodegeneratif dan


inflamasi pada MS. (Dendrou CA,
2015)
5. Clinical Manifestations

Subtipe Klinis

Tabel 1. Tipe-tipe MS (Loma I, 2011)

Most common type, accounts for approximate 85% of


Relapsing/Remitting cases. Characterized by discrete attacks that evolve
Multiple Sclerosis over days to weeks followed by some decree of
(RRMS) recovery over weeks to months. In between attacks,
the patient has no worsening neurological function.
Secondary Progressive Characterized by initial relapses, followed by
Multiple Sclerosis gradual neurological deterioration not associated
(SPMS) with acute attacks.
Primary Progressive Characterized by steady functional decline from the
Multiple Sclerosis onset of the disease. No relapses ever.
(PPMS)
Characterized by steady functional decline from onset
Progressive Relapsing
of the disease with later superimposed acute attacks.
Multiple Sclerosis
PRMS and PPMS cannot be distinguished during
(PRMS)
early stages, until the relapses occur

Tabel 2. Gejala dan tanda dari MS. (Aminoff MJ, 2015)


Tanda Persen
Absent abdominal
81
reflexes
Gejala Persen Hyperreflexia 76
Paresthesia 37
Lower extremity ataxia 57
Gait disorder 35
Lower extremity Extensor plantar
54
weakness or 17 responses
incoordination
Impaired rapid
49
Visual loss 15 alternating movements
Upper extremity Impaired vibratory
47
weakness or 10 sense
incoordination Optic neuropathy 38
Diplopia 10 Nystagmus 35
Impaired joint position
33
sense
Intention tremor 32
Spasticity 31
Impaired pain or
22
temperature sense
Dysarthria 19
Paraparesis 17
Internuclear
11
ophthalmoplegia
6. Diagnostic Studies

Gambar 6. Protokol standar MRI kepala


pada MS.
a | Pre-contrast, b | axial T1-weighted and c | dualecho T2-weighted
sequences, d
| contrast-enhanced sagittal, e | axial 2D T2-weighted fluid-attenuated
inversion recovery (FLAIR) and f | axial T1-weighted sequences. (Rovira
À, 2015)
Gambar 7. Medula spinalis servikal irisan
sagital.
a | Fast spin-echo proton-density,b | T2-weighted and c | short-tau
inversion recovery (STIR) MRI sequences. (Rovira À, 2015)

Tabel 6. Biomarker pada MS (Comabella M, 2014)

Category Body fluid Biomarker test


Natalizumab response
Anti-natalizumab
biomarker for an Serum ELISA
antibodies
adverse effect
CPE, luciferase
Neutralising Interferon-beta
Serum*, PBMCs gene-reporter
antibodies response biomarker
assay
IgG oligoclonal
Diagnostic CSF, serum EF/IB
bands
IgG index Diagnostic CSF, serum Formula‡
Anti-aquaporin-4 HC, CBA,
Diagnostic Serum*, CSF
antibodies ELISA
Natalizumab response
Anti-JC virus
biomarker for an Serum*, plasma ELISA
antibodies
adverse effect
Fingolimod-response
Anti-VZV
biomarker for an Serum*, plasma ELISA
antibodies
adverse effect

Gambar 7. Diagnostik pada LCS. (Mary, AM. 2012)


Evoked Potentials (EPs)

Gambar 8. Visual evoked response (VER) (Mary,


AM. 2012)

Gambar 9. Somatosensory evoked responses (SER) (Mary,


AM. 2012)
Table 1. The 2017 McDonald Criteria for diagnosis of multiple
sclerosis in patients with an attack at onset.

Table 2. 2017 McDonald criteria for diagnosis of multiple


sclerosis in patients with a disease course characterised by
progression from onset (primary progressive multiple sclerosis)

7. Differential Diagnosis

Tabel 3. Diagnosis banding pada MS. (Joel O, 2006)

• Balo disease
MS variants • Schilder disease
• Marburg disease
Overlap • Optic neuritis
syndromes • Transverse myelitis
• Devic’s disease
• Harding disease (Leber variant)
• Acute/monophasic disseminated encephalomyelitis (ADEM)
Other
demyelinating • Noninflammatory diseases such as PML, central pontine
disease myelinolysis, and B12 deficiency
• Common leukodystrophies such as adrenoleukodystrophy
(ALD)/metachromatic leukodystrophy (MLD) and Krabbe
disease
• Vasculitis
• Connective tissue disease (Sjögren syndrome, SLE)
Other • Neurosarcoid
inflammatory • Whipple disease
diseases • Behçet disease
• Infective disease (human T-cell lymphotropic virus 1
[HTLV1], tertiary Lyme disease)
Malignancy and genetic (HSP)
Vascular disease, including antiphospholipid syndrome

8. Prognosis
9. Treatment

Tabel 9. Obat untuk prevensi dan relap pada MS. (Kes, VB, 2012)

Drugs Dose Level of


Evidence

Methylprednisolone 0.5-1 g per day i.v.(3-7 days) I


0.5-1 mg/kg body weight in
Prednisone taperingdoses; after 3-6 weeks, IV
Treatment
5-10 mg maintenance dose
of relapses
2.0-0.4 g/kg body weight 2-5
IVIG III
days
Plasma exchange 1-7 times every other day IV
Glatiramer acetate 20 mcg per day I
Avonex 6 MIU once per week,
First line Interferon beta 1a
Betaferon9.6 MIU every other
therapies and interferon beta I
day, Rebif 22 mcgor 44 mcg
1b
twice weekly
Fingolimod 0.5 mg per os per day I
Natalizumab 300 mg i.v. every 4 weeks II
2.5-3 mg/kg body weight per
Azathioprine IV
day; 1.5-2.5 maintenance dose
1-5 mg/kg twice per day
1 g i.v. every month for 6-12
months,then every 5 weeks
Cyclophosphamide II
during 2nd year andevery 6
weeks during 3rd year of
Second line application
therapies 20 mg +1 g
methylprednisolone
oncemonthly or once in 3
Mitoxantrone months or 2-3x20mg per I
month followed by 10 mg
once in3 months till
cumulative dose
Mycophenolate 1 g twice daily
IV
mofetil

Gambar 11. Mekanisme obat injeksi pada MS. (Oh J, 2013)

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