CASE OF THE MONTH
Mon 25th March 2013
Dr. Mohamed Saher Hashem
Dr. Alaa El Mazny
Dr. Amr Hasan M.D.
DR AMR HASAN AL HASANY
Personal History
• S.E., 19 years old female patient, from
Benisuef , college student, married for 1
month , right handed , NSHMI.
• Menstrual h/o : menarche at the age of 12
with regular cycles occurring every 28 days
and lasts for 4 days.
DR AMR HASAN AL HASANY
Complaint
Recurrent attacks of weakness of both U.L.s
and L.L. s of 5 months duration.
DR AMR HASAN AL HASANY
Family History
• No Similar Condition in her family.
• -ve consanguinity.
DR AMR HASAN AL HASANY
Past History
• -ve for
Diabetes , Hypertension
Previous surgical operations
Drug allergy
Chronic drug intake
Blood Transfusion
Other System Affection
DR AMR HASAN AL HASANY
Present History
Oct 2012
Diarrhea : Watery
Vomiting :
2-3 times / day not preceded by nausea
abdominal pains and not related to meals , it
occurred for 2 days.
DR AMR HASAN AL HASANY
Present History
1 week later:
Weakness :
Acute onset regressive course affecting both upper and lower
limbs that occurred simultaneously over 2 days UL = LL , RT > LT
and P=D.
Patient felt her limbs flail.
Patient became bedridden within 3 days.
DR AMR HASAN AL HASANY
Present History
Tingling of upper and lower limbs
suggesting
of
deep
D>P , no symptoms
sensory
affection
Urine Retention.
Abnormal Involuntary Movement UL & LL during
movement and increasing on reaching the target.
Accumulation of food behind cheek and inability
to close both eyes properly .
DR AMR HASAN AL HASANY
Examination
I. General :
1) Vital Signs : Pulse 100
Temp 37.5
2) Chest
Abdomen
LN
Joints
BP 130/70
RR 25
Heart
Skin
NL
II. Mental State :
MMSE : 30/30
III. Speech : Normal
DR AMR HASAN AL HASANY
IV. Cranial Nerves :
Bilateral LMN Facial
Nystagmus in all directions of gaze and in
the primary position
V. Motor System
.Tone : NL
Power : G 3-4
UL = weakness E>F
LL = weakness F>E
.Reflexes -- AREFLXIA (UL – LL)
-- Planter : BIL. EXTENSOR
-- Abdominals : Preserved
DR AMR HASAN AL HASANY
VI. Coordination :
+ve finger to nose
+ve heel to knee
VII. Sensory :
Superficial
Deep ( vibration & position) both intact
DR AMR HASAN AL HASANY
Localization !!!
DR AMR HASAN AL HASANY
UMNL Vs LMNL
DR AMR HASAN AL HASANY
UMNL Vs LMNL
UMNL
Nystagmus
5% oF LGB
Bilateral extensor plantar
5% of LGB
Distribution of weakness
Retention of urine
Ataxia
May be Radicular
distribution
5% of LGB
May be sensory ataxia
( but nystagmus !!!)
DR AMR HASAN AL HASANY
UMNL Vs LMNL
LMNL
Flaccid weakness
Shock stage
Bilateral symmetrical
LMN facial palsy
Distal parasthesia
!!!!!!!
History of antecedent
infection
!!!!!!!
Subjective !!!!
DR AMR HASAN AL HASANY
What is the most
appropriate initial
investigation for this
Patient ?
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
EMG and NC study
• No detectable abnormality
DR AMR HASAN AL HASANY
What is the most
Probable diagnosis
for this Patient ?
DR AMR HASAN AL HASANY
LGB
WHAT ABOUT UMNL SIGNS
DR AMR HASAN AL HASANY
What is the most
appropriate treatment
for this Patient ?
DR AMR HASAN AL HASANY
• She received 6 sessions of plasma exchange ->
nearly complete recovery of her symptoms ,
patient discharged ambulant
with out
support
with physiotherapy after discharge
complete recovery of her symptoms
DR AMR HASAN AL HASANY
Jan 2013
Vomiting :
2-3 times/day for 2 days not realted to meals.
No diarrhea , abdominal pains or nausea.
DR AMR HASAN AL HASANY
Present History
3 days later:
The Same attack exactly with the following differences
1- Reflexes are preserved in U.L. and brisk in
L.L.
2- Bulbar palsy ( TRUE , TRANSIENT).
3- No sphincteric troubles.
4- Respiratory distress ( needed ICU admission)
DR AMR HASAN AL HASANY
Examination
I. General :
1) Vital Signs
2) Chest
LNS
pulse 80
RR paradoxical
BP 100/60
Temp 37.5
Abdomen Heart
Joints
Skin
NL
II. Mental : MMSE 30/30
III. Speech : Staccato
DR AMR HASAN AL HASANY
Examination
IV. Cranial Nerves :
Nystagmus in all directions of gaze with in the
primary position
NAD
V. Motor System
Tone = Nl
Power= D>P
LT > RT
G 2-3
3-4
Reflexes : UL Grade 2
LL Grade 3 (knee and ankle)
Plantar : BIL. EXTERNAL PLANTAR
Abdominals : Lost
DR AMR HASAN AL HASANY
Examination
VI. Coordination : +ve finger to nose
+ve heel to knee
RT
LT : couldn’t be assessed VII. Sensory : Deep and Superficial
DR AMR HASAN AL HASANY
Localization !!!
DR AMR HASAN AL HASANY
UMNL Vs LMNL
DR AMR HASAN AL HASANY
UMNL Vs LMNL
UMNL
Nystagmus
Bilateral extensor plantar
Distribution of weakness
Retention of urine
Ataxia
Brisk reflexes
Respiratory distress
LMNL
Flaccid weakness
Bilateral symmetrical
LMN facial palsy
Distal parasthesia
History of antecedent
infection
Respiratory distress
True bulbar
DR AMR HASAN AL HASANY
Should we revise the
diagnosis?
Is there any additional
investigation to be
done?
DR AMR HASAN AL HASANY
Due to her respiratory embarrasement >>>>
PE was done.
Patient Received 4 sessions of plasma exchange
>>>> complete recovery of her symptoms.
DR AMR HASAN AL HASANY
Should we revise the
diagnosis?
Is there any additional
investigation to be
done?
DR AMR HASAN AL HASANY
Special Appreciation to
Dr Alaa El Mazny
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
MRI Cervical spine
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
MRI Brain
DR AMR HASAN AL HASANY
Can we change
diagnosis to M.S. ?
DR AMR HASAN AL HASANY
Dissemination in time
Dissemination in space
BETTER EXPLANATION ?????
DR AMR HASAN AL HASANY
The Patient back to normal !!!!
DR AMR HASAN AL HASANY
7 days after marriage
The 3RD attack
DR AMR HASAN AL HASANY
Feb 2013
Sensory :
Acute onset regressive course of tingling and numbness affecting RT
UL&LL’s
2 days later
Weakness :
RT UL& LL , LL>UL , D>P , patient felt her limbs flail with no muscle
wasting nor twitches
Patient was initially bed ridden for 2 days then she become Ambulant
with MAX. support
Cranial nerves
Double vision on looking to left side.
NO Sphincteric troubles
DR AMR HASAN AL HASANY
• In view of her MRI brain her traeating
Neurologist prescribed her ACTH injection
just before readmission , upon which she
improved and her plegic U.L.
Paretic and
she became ambulant with max support
instead of being bed ridden.
• Then
re admission for the third time.
DR AMR HASAN AL HASANY
Examination
I.General 1) Vital Signs
BP100/60
Pulse 80
2) Chest
LNS
II.Mental State :
MMSE 30/30
III. Speech : Staccato
RR 25
Temp 37
Abdomen
Joints
Heart
Skin NL
DR AMR HASAN AL HASANY
III. Cr Nerves :
6th nerve palsy (left eye)
No other abnormality detected
IV.Motor : Tone & Status NL
power = RT
LT
UL 3-4
5
LL D:2-3
5
P:3-4
Reflexes :
UL : G 2
LL : G 3
Patellar and Adductor reflexes present.
BIL. EXTENSOR PLANTAR
Abdominals : Lost
DR AMR HASAN AL HASANY
Coordination : + ve finger to nose
LT
+ ve heel to knee
Rt couldn’t be assessed
Sensory : Superficial and Deep intact
DR AMR HASAN AL HASANY
Patient was admitted afterwards receiving :
5 grams of Methyl Prednisolone marked
improvement
now she is completely
symptom free
DR AMR HASAN AL HASANY
INVESTIGATIONS
CBC
Na , K
Kidney
Liver
Urine analysis
NL
NL
NL
NL
NL
Glucose
PT - PC - PTT- INR
NL
NL
DR AMR HASAN AL HASANY
• ESR : 1st Hour = 33 mm/hr
2nd Hour = 68 mm/hr
(nl up to 20)
(nl up to 20)
• ANA : -ve
• Lupus AntiCoagulant : NL (37.4)
(34-40)
• B2 Microglobulin : 959.1 ug/L
(850-1150)
DR AMR HASAN AL HASANY
Neuro-physiological Studies :
VEP : no detectable abnormalities bilaterally
DR AMR HASAN AL HASANY
Imaging
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
CSF Examination
• Ig G Index Ratio
• OCB
DR AMR HASAN AL HASANY
CSF Examination
• Ig G Index Ratio
3.1
(nl up to 0.7)
• OCB
POSITIVE
DR AMR HASAN AL HASANY
Should we establish
diagnosis of M.S.?
Is there any additional
investigation to be
done?
DR AMR HASAN AL HASANY
Better explanation ??
• CNS LYMPHOMA ?
• SARCOIDOSIS ?
• BEHCET DISEASE ?
• CEREBRAL ANGIITIS ?
DR AMR HASAN AL HASANY
What is the most
appropriate long term
Treatment
for this Patient ?
DR AMR HASAN AL HASANY
What is interesting in this case if
diagnosis of M.S. is established
•
•
•
•
LGB like presentation.
Vomiting that precede the attacks.
Initially normal MRI brain.
Marvelous response to PE.
DR AMR HASAN AL HASANY
• ALAA SLIDES
DR AMR HASAN AL HASANY
Protocol for Management of
Multiple Sclerosis
(K.A.M.S. Protocol)
Kasr Al Aini Multiple Sclerosis unit
Multiple Sclerosis Research Group
Neurology Department
Faculty of Medicine
Cairo University DR AMR HASAN AL HASANY
K.A.M.S. Protocol
Diagnosis of M.S.
Treatment of M.S.
Treatment of Relapsing Remitting MS
Treatment of a relapse
Treatment in between attacks (DMT)
When to start DMT?
How to choose DMT?
How to follow up treatment response?
When to switch to another line?
Treatment of Secondary Progressive MS
Treatment of Primary Progressive MS
Treatment of Neuromyelitis optica (NMO)
Treatment of Clinically Isolated Syndrome
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
Diagnosis of MS includes
To prove it is M.S
To exclude other
diagnoses
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To prove M.S.
Clinical:
•History and examination.
•Evidence of CNS lesions dissemination in space and time.
Paraclinical
1.Neuroimaging.
2.Evoked potentials.
3.CSF analysis
What are the criteria used in diagnosis?
criteria for the diagnosis of Relapsing remitting M.S.
Mc Donald 2010 MRI criteria for dissemination in space (DIS) and time
(DIT) 4 :
DR AMR HASAN AL HASANY
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To prove M.S.
Clinical:
•History and examination.
•Evidence of CNS lesions dissemination in space and time.
Paraclinical
1.Neuroimaging.
2.Evoked potentials.
3.CSF analysis
What are the criteria used in diagnosis?
criteria for the diagnosis of Relapsing remitting M.S.
Mc Donald 2010 MRI criteria for dissemination in space (DIS) and time
(DIT) 4 :
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
A. Clinical Red Flags
Optic neuritis: Absence of pain, retinal exudates or hemorrhages, severe disc
swelling, bilateral involvement, no visual recovery after 1 month, uveitis.
Brainstem syndrome: Hyperacute onset, vascular territory distribution (e.g. lateral
medullary syndrome), age >50 years, isolated trigeminal neuralgia, fluctuating
ocular/bulbar weakness, non-remitting symptoms, fever, meningismus, complete
external ophthalmoplegia, third nerve palsy, focal dystonia or torticollis.
Marked LMN signs: Areflexia, proximal weakness, bilateral LMN facial palsy, cauda
equina lesion.
Spinal cord syndrome:
Hyperacute onset or insidiously progressive, complete
transverse myelitis, sharp sensory level, Radicular pain, failure to remit, anterior
spinal artery distribution (sparing posterior columns only), complete Brown-Sequard
syndrome.
Cerebral hemisphere: obtundation, confusion, cortical blindness, dementia, aphasia,
extrapyramidal features, seizures.
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
A. Clinical Red Flags
Optic neuritis: Absence of pain, retinal exudates or hemorrhages, severe disc
swelling, bilateral involvement, no visual recovery after 1 month, uveitis.
Brainstem syndrome: Hyperacute onset, vascular territory distribution (e.g. lateral
medullary syndrome), age >50 years, isolated trigeminal neuralgia, fluctuating
ocular/bulbar weakness, non-remitting symptoms, fever, meningismus, complete
external ophthalmoplegia, third nerve palsy, focal dystonia or torticollis.
Marked LMN signs: Areflexia, proximal weakness, bilateral LMN facial palsy, cauda
equina lesion.
Spinal cord syndrome:
Hyperacute onset or insidiously progressive, complete
transverse myelitis, sharp sensory level, Radicular pain, failure to remit, anterior
spinal artery distribution (sparing posterior columns only), complete Brown-Sequard
syndrome.
Cerebral hemisphere: obtundation, confusion, cortical blindness, dementia, aphasia,
extrapyramidal features, seizures.
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
B. Laboratory and radiological Red Flags
CSF features atypical for MS at initial presentation: White blood cells > 50, protein >
80mg/dl, absence of oligoclonal bands (by isoelectric focusing technique).
MRI features atypical for MS at initial presentation:
Brain MRI: Normal, small lesions < 3 mm, subcortical location, prominent infratentorial
involvement, prominent grey matter involvement (basal ganglia), symmetric confluent
hemispheric white matter involvement, hydrocephalus, severe cerebellar/brain stem
atrophy, no callosal/periventricular lesions, hemorrhagic lesions, cortical infarcts,
meningeal enhancement, simultaneous enhancement of all lesions
Spinal MRI: Large lesion spanning multiple segments (>2), severe swelling, full
thickness lesions, leptomenengial enhancement, T1 hypointense lesions, diffuse
abnormalities in the posterior columns
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
C. Additional tests to be considered based on red flags
CNS Inflammatory/Autoimmune Disease: ANA,CRP, Anti DS
DNA,C3,C4, ANCA panel, Chest CT, eye exam, conjunctival biopsy,
Pathergy skin test (Behcet's), Skin biopsy if suspicious rash
present, CTA, angiogram .
CNS Infection: Brucella antibodies, HIV test, HTLV1, and CSF
antibodies (if isolated myelopathy with a lesion on spinal MRI),
ESR, , small bowel biopsy for whipple .
CNS Neoplasm/ Infiltrative Disorder: CSF cytology and flow
cytometry, CXR, CT Chest\ Abdomen\Pelvis, Pelvic ultrasound,
Mammogram, LDH, skeletal series, bone scan,DR
Brain
AMR biopsy.
HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
C. Additional tests to be considered based on red flags
CNS vasculopathy/Ischemic Disease: Notch3 mutations in
CADASIL , MRA, CTA, standard angiogram, thrombophilia panel,
Lupus anticoagulant .
Nutritional deficiency/Toxicity: Vitamin B12, Copper and Zinc
Levels, Ceruloplasmin, Folate, Heavy metal screen.
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
To exclude other diagnoses:
C. Additional tests to be considered based on red flags
Dysmyelinating/ Metabolic Disorders: Lumbar puncture,
EMG/NCVs, biochemical studies, buccal or rectal mucosa biopsy
for electron microscopy if neuronal ceroid lipofuscinosis (NCL)
suspected, skin biopsy for fibroblast cultures (enzyme assays), or
peripheral blood smear (vacuolated monocytes in NCL), Brain
biopsy (rarely needed): fingerprint profiles, curvilinear and
rectilinear bodies by E.M. in NCL oligodendrocytes; diffuse white
matter gliosis by light microscopy in NCL.
Urine/blood for biochemical studies, including levels of: WBC
arylsulfatase A, Very long chain fatty, Fasting arterial lactate,
Quantitative plasma amino acid and Urine organic acid analyses.
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
Treatment of Relapsing Remitting MS:
Definition of a relapse.
Treatment of a relapse.
Treatment in between attacks (DMT).
When to start DMT?
How to choose DMT?
How to follow up treatment response?
When to switch to another line?
DR AMR HASAN AL HASANY
K.A.M.S. Protocol
K.A.M.S. Protocol Team
Prof.Dr. Sherif Hamdy
Ass.Prof.Dr. Nirmeen Adel
Dr. Marwa Farghaly
Dr. Amr Hasan
Dr. Mohammed Hegazy
DR AMR HASAN AL HASANY
Thank You
DR AMR HASAN AL HASANY