Dr. Md Rashedul Islam
FCPS, MRCP(UK)
Registrar, Neurology, BIRDEM
A 57 years old diabetic right handed gentleman,
hailing from Mirpur, got admitted in BIRDEM
General Hospital on 11th
March,14 with the
complaints of-
• Double vision for 6 days
• Difficulty in walking for 4 days
According to the statement of the patient, he
was reasonably well 6 days back. Then he
developed double vision which was sudden
on onset, constant, more marked on eye
movement & not associated with diuranal
variation. He also had complaints of
headache which was sudden on onset, global,
dull aching in nature, mild to moderate in
severity. It was not associated with radiation,
vomiting. There was no exacerbating factors
& it was relieved with medication.
• He also had complaints of difficulty in walking
for last 4 days which was gradual on onset due
weakness of lower limbs progressing to upper
limbs rapidly. It was associated with
numbness of limbs. There was no associated
back pain, vertigo on standing, palpitation,
shortness of breath, swallowing difficulties,
slurring of speech. He doesn't give h/o cough
& diarrhea.
H/O Present illness
He consulted an endocrinologist for these
problems as he assumed that it might be due
to high blood sugar. He was advised for CT
scan & subsequent MRI of brain &
diagnosed as a case of DMT2, Acute
ischaemic stroke, cranial polyangitis due to
diabetes with DPN. He was treated with
insulin, aspirin, atorvastatin & vtamin B
complex. He got admitted in Neurology,
BIRDEM as he was not improving.
CT Scan of Brain
CT Scan of Brain
H/O past illness:
Nothing contributory
Socioeconomic history:
He belongs to a middle class family
Personal history:
He is non alcoholic, non smoker
Family history:
Nothing significant
Treatment history:
Insulin
Tab. Vit B complex
Tab. Aspirin
Tab. Atorvastatin
General examination:
Appearance: ill looking, anxious
Built: average
Decubitus: on choice
Anaemia
Jaundice
Cyanosis
Oedema
Dehydration
Clubbing
Koilonychia
Leukonychia
Absent
General examination:
Neck vein: not engorged
Thyroid: not enlarged
Lymph node: not palpable
Skin pigmentation & body hair distribution: normal
Pulse: 78 b/min
BP: 120/70 mmHg
Temp:98 F
RR: 16 breaths/min
• Higher psychic function : Conscious, Oriented
• Speech: Normal
• Cranial nerves :
Bilateral complete opthalmoplegia with right
sided lower motor type VII nerve palsy
• Fundus: Normal
• GCS: 15/15
NERVOUS SYSTEM EXAMINATION
Muscle Rt. UL Lt. UL Rt. LL Lt. LL
Bulk Normal Normal Normal Normal
Tone Decreased Decreas
ed
Decreased Decreased
Power 3/5 3/5 3/5 3/5
Involuntary
movement
Absent Absent Absent Absent
MOTOR FUNCTION:
Reflex B T S K A Abd Plantar
Right Abs
ent
Abs
ent
Abse
nt
Abse
nt
Ab
sen
t
Absent Flexor
Left Abs
ent
Abs
ent
Abse
nt
Abse
nt
Ab
sen
t
Absent
Flexor
Sensory system:
Pain Temp Touch Vibratio
n
Position
sense
Right upper
limb
Intact
Right lower
limb
Left upper
limb
Left lower
limb
• Sign of Meningeal irritation - Absent
• Cerebellar sign : Absent
• Gait: Ataxic
Systemic examinations
Other systemic examination was normal
A 57years old diabetic gentleman got admitted
in neurology with the complaints of double
vision which was sudden on onset, more
marked on eye movement & not associated
with diurnal variation. He also had complaints
of headache which was sudden on onset,
global, dull aching in nature, mild to moderate
in severity. It was not associated with radiation,
vomiting.
Salient feature
Salient feature
He also had complaints of difficulty in walking
for last 4 days which was gradual on onset due
to weakness of lower limbs progressing to upper
limbs rapidly. It was associated with numbness
of limbs. There was no associated back pain,
vertigo on standing, palpitation, shortness of
breath, swallowing difficulties, slurring of
speech. He doesn't give h/o cough & diarrhea.
• On examination, he was anxious, ill looking,
bilateral complete opthalmoplegia with right
sided lower motor type VIIth nerve palsy,
Generalized hypotonia, diminished muscle
power, generelized areflexia with bilateral
planter flexor. All modalities of sensation were
intact with ataxic gait.
Provisional diagnosis
• Guillain-Barre Syndrome(Miller Fisher
Variant)
• Diabetes Mellitus Type 2
• Leukoaraiosis / Periventricular white
matter disease
Differential diagnosis
• Mononeuritis cranial multiplex due to?
Vasculitis/Diabetes
Investigations
CBC:
Hb % - 14.2
WBC -6800 cu/mm
Neu-65 %
Lymph- 30%
Mono -3 %
Eosino- 1.1%
Platelet- 195000
ESR- 30mm in 1st
hour
S. Electrolytes
Na-137 mmol/l
K-4.5 mmol/l
Cl: 106 mmol/l
HCO3: 26 mmol/l
Ca- 9.3 mmol/l
Mg- 0.9 mmol/l
Phosphate-3.7
Lipid profile:
TG: 176 mg/dl
T. Chol : 164 mg/dl
LDL: 95 mg/dl
HDL:36 mg/dl
LFT:
ALT: 34 iu/L
AST: 37 iu/L
RFT:
S. Creatinine: 0.8mmol/l
S Urea: 29 mmol/l
HbA1c: 7.2%
Sugar - Nil
Albumin – Nil
Ketone- Nil
Epi. cell: A few /HPF
Pus cell: 1-2 /HPF
RBC: Nil
URINE R/M/E
Chest X-Ray
NORMAL
ECG
Normal
Nerve conduction study
• Pure motor polyneuropathy-
• Possibilities are:
• AMAN
• Other motor polyradiculoneuroapthy
CSF study
• Appearance: clear
• Protein: 66g/L
• Sugar: 4.2mmol/L( Corresponding blood
glucose-6.8 mmol/l)
• Cell count:
• Total WBC : Nil
• Total RBC: Nil
• Bacterial antigen: Negative
Final diagnosis:
• Guillain-Barre Syndrome(Miller Fisher Variant)
• Diabetes Mellitus Type 2
• Leukoaraiosis / Periventricular white matter
disease
Treatment:
Short acting insulin
I/V immunoglobulin
Daily physiotherapy
Vitamin B
Patient was counseled about Course and
prognosis of the disease
Hospital course
• He showed significant improvement with
treatment.
• Gait & opthalmoplegia was improved
Follow UP
Patient was advised to follow up in Neurology
after 1 month for further clinical evaluation &
management.
Miller fisher syndrome

Miller fisher syndrome

  • 1.
    Dr. Md RashedulIslam FCPS, MRCP(UK) Registrar, Neurology, BIRDEM
  • 2.
    A 57 yearsold diabetic right handed gentleman, hailing from Mirpur, got admitted in BIRDEM General Hospital on 11th March,14 with the complaints of- • Double vision for 6 days • Difficulty in walking for 4 days
  • 3.
    According to thestatement of the patient, he was reasonably well 6 days back. Then he developed double vision which was sudden on onset, constant, more marked on eye movement & not associated with diuranal variation. He also had complaints of headache which was sudden on onset, global, dull aching in nature, mild to moderate in severity. It was not associated with radiation, vomiting. There was no exacerbating factors & it was relieved with medication.
  • 4.
    • He alsohad complaints of difficulty in walking for last 4 days which was gradual on onset due weakness of lower limbs progressing to upper limbs rapidly. It was associated with numbness of limbs. There was no associated back pain, vertigo on standing, palpitation, shortness of breath, swallowing difficulties, slurring of speech. He doesn't give h/o cough & diarrhea.
  • 5.
    H/O Present illness Heconsulted an endocrinologist for these problems as he assumed that it might be due to high blood sugar. He was advised for CT scan & subsequent MRI of brain & diagnosed as a case of DMT2, Acute ischaemic stroke, cranial polyangitis due to diabetes with DPN. He was treated with insulin, aspirin, atorvastatin & vtamin B complex. He got admitted in Neurology, BIRDEM as he was not improving.
  • 6.
  • 7.
  • 12.
    H/O past illness: Nothingcontributory Socioeconomic history: He belongs to a middle class family Personal history: He is non alcoholic, non smoker
  • 13.
    Family history: Nothing significant Treatmenthistory: Insulin Tab. Vit B complex Tab. Aspirin Tab. Atorvastatin
  • 14.
    General examination: Appearance: illlooking, anxious Built: average Decubitus: on choice Anaemia Jaundice Cyanosis Oedema Dehydration Clubbing Koilonychia Leukonychia Absent
  • 15.
    General examination: Neck vein:not engorged Thyroid: not enlarged Lymph node: not palpable Skin pigmentation & body hair distribution: normal Pulse: 78 b/min BP: 120/70 mmHg Temp:98 F RR: 16 breaths/min
  • 16.
    • Higher psychicfunction : Conscious, Oriented • Speech: Normal • Cranial nerves : Bilateral complete opthalmoplegia with right sided lower motor type VII nerve palsy • Fundus: Normal • GCS: 15/15 NERVOUS SYSTEM EXAMINATION
  • 18.
    Muscle Rt. ULLt. UL Rt. LL Lt. LL Bulk Normal Normal Normal Normal Tone Decreased Decreas ed Decreased Decreased Power 3/5 3/5 3/5 3/5 Involuntary movement Absent Absent Absent Absent MOTOR FUNCTION:
  • 19.
    Reflex B TS K A Abd Plantar Right Abs ent Abs ent Abse nt Abse nt Ab sen t Absent Flexor Left Abs ent Abs ent Abse nt Abse nt Ab sen t Absent Flexor
  • 20.
    Sensory system: Pain TempTouch Vibratio n Position sense Right upper limb Intact Right lower limb Left upper limb Left lower limb
  • 21.
    • Sign ofMeningeal irritation - Absent • Cerebellar sign : Absent • Gait: Ataxic
  • 22.
  • 23.
    A 57years olddiabetic gentleman got admitted in neurology with the complaints of double vision which was sudden on onset, more marked on eye movement & not associated with diurnal variation. He also had complaints of headache which was sudden on onset, global, dull aching in nature, mild to moderate in severity. It was not associated with radiation, vomiting. Salient feature
  • 24.
    Salient feature He alsohad complaints of difficulty in walking for last 4 days which was gradual on onset due to weakness of lower limbs progressing to upper limbs rapidly. It was associated with numbness of limbs. There was no associated back pain, vertigo on standing, palpitation, shortness of breath, swallowing difficulties, slurring of speech. He doesn't give h/o cough & diarrhea.
  • 25.
    • On examination,he was anxious, ill looking, bilateral complete opthalmoplegia with right sided lower motor type VIIth nerve palsy, Generalized hypotonia, diminished muscle power, generelized areflexia with bilateral planter flexor. All modalities of sensation were intact with ataxic gait.
  • 26.
    Provisional diagnosis • Guillain-BarreSyndrome(Miller Fisher Variant) • Diabetes Mellitus Type 2 • Leukoaraiosis / Periventricular white matter disease
  • 27.
    Differential diagnosis • Mononeuritiscranial multiplex due to? Vasculitis/Diabetes
  • 28.
    Investigations CBC: Hb % -14.2 WBC -6800 cu/mm Neu-65 % Lymph- 30% Mono -3 % Eosino- 1.1% Platelet- 195000 ESR- 30mm in 1st hour
  • 29.
    S. Electrolytes Na-137 mmol/l K-4.5mmol/l Cl: 106 mmol/l HCO3: 26 mmol/l Ca- 9.3 mmol/l Mg- 0.9 mmol/l Phosphate-3.7
  • 30.
    Lipid profile: TG: 176mg/dl T. Chol : 164 mg/dl LDL: 95 mg/dl HDL:36 mg/dl LFT: ALT: 34 iu/L AST: 37 iu/L RFT: S. Creatinine: 0.8mmol/l S Urea: 29 mmol/l HbA1c: 7.2%
  • 31.
    Sugar - Nil Albumin– Nil Ketone- Nil Epi. cell: A few /HPF Pus cell: 1-2 /HPF RBC: Nil URINE R/M/E
  • 32.
  • 33.
  • 34.
    Nerve conduction study •Pure motor polyneuropathy- • Possibilities are: • AMAN • Other motor polyradiculoneuroapthy
  • 35.
    CSF study • Appearance:clear • Protein: 66g/L • Sugar: 4.2mmol/L( Corresponding blood glucose-6.8 mmol/l) • Cell count: • Total WBC : Nil • Total RBC: Nil • Bacterial antigen: Negative
  • 36.
    Final diagnosis: • Guillain-BarreSyndrome(Miller Fisher Variant) • Diabetes Mellitus Type 2 • Leukoaraiosis / Periventricular white matter disease
  • 37.
    Treatment: Short acting insulin I/Vimmunoglobulin Daily physiotherapy Vitamin B Patient was counseled about Course and prognosis of the disease
  • 38.
    Hospital course • Heshowed significant improvement with treatment. • Gait & opthalmoplegia was improved
  • 39.
    Follow UP Patient wasadvised to follow up in Neurology after 1 month for further clinical evaluation & management.