Miller fisher syndrome

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, anxiousBuilt: average Decubitus: on choiceAnaemiaJaundiceCyanosisOedemaDehydrationClubbingKoilonychiaLeukonychia

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 Decreased

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 Absent

Absent

Absent

Absent

Absen

t

Absent Flexor

Left Absent

Absent

Absent

Absent

Absen

t

Absent

Flexor

Sensory system:

Pain Temp Touch Vibration

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/lCl: 106 mmol/lHCO3: 26 mmol/lCa- 9.3 mmol/lMg- 0.9 mmol/lPhosphate-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.