A 58-year-old lady presented to ER on 24th April 2023 at around 7.30 pm with
- History of low back pain x 2 days followed by sudden onset of giddiness, headache,
- double vision involving both eyes with photosensitivity, and
- bilateral numbness of hand and fingers, perioral numbness
- decreased sensation over both feet
PAST MEDICAL HISTORY:
- She is known Diabetic ,
- Guillian barre syndrome-treated 28 yrs back,
- cervical spondylosis and anxiety disorder
INITIAL VITALS & EXAMINATION DONE AT ER:
She was conscious, oriented, alert, afebrile. Vitals were within normal limits.
Systemic Examination :
Respiratory system: B/L air entry present, NVBS
CVS: S1,S2 heard, no murmur, JVP normal
Per abdomen: soft, non-tender, no org, BS+
Central nervous system: GCS- 15/15, b/l pupil reacting to light 3mm sluggish
EOM– bilateral lateral rectus palsy- complete restriction
Bilateral medial rectus palsy- partial restriction
Upward and downward gaze restriction
Bilateral partial ptosis
No pronator drift
| RIGHT | LEFT | |
| POWER BOTH UL AND LL | 5/5 | 5/5 |
| REFLEXES | ||
| Biceps | + | reduced |
| Triceps | + | reduced |
| Supinator | + | reduced |
| Knee | absent | absent |
| Ankle | absent | absent |
| Plantar | mute | mute |
Initial workup and investigations:
MRI BRAIN with MRA and MRV: no evidence of hemorrhage, mass lesion, demyelination.
Blood Investigation:
- Hb- 13.8 WBC- 10,500 platelet- 3,98,000 CRP-75
- UREA-28, CREAT- 0.7
- NA-136, K-4.5, CL-98
- S.CORTISOL- 13.4
S.ganglioside, lyme serology, scrub typhus were all negative
EEG– slow wave activity over anterior region
NCS– both motor and sensory axonal demyelination
Initially patient was admitted in ward during late evening . On the same day over night patient progressed to bilateral complete ptosis, loss of voice and loss of power in both upper limb with preserved muscle power in knee and ankle.
A diagnosis of Guillian barre syndrome- MILLER FISHER variant suspected.
With acute onset symptoms and based on clinical examination she was started on IV immunoglobulin (immunogrel) 30 gram/day x 5 days. Meanwhile she started to desaturate and was immediately shifted to Neuro ICU. There she was maintained with NIV support. Her blood reports showed high white cell count, CRP, procalcitonin, high glucose level, ABG- severe metabolic acidosis and CT chest shows aspiration pneumonia so started on IV broad spectrum antibiotics and DKA correction done.
On Day-2 There was a drop in GCS – she was intubated after obtaining consent.
On Day-4 In view of progression of her symptoms and prolonged ventilatory assistance she underwent tracheostomy.
On Day-5 She developed complete quadriplegia with absence of light and cough reflex.
Due to rapid progression of her symptoms , multidisciplinary discussion was made and decided to initiate plasmapheresis.
On DAY-7 Right IJV was secured and first cycle of PLEX was initiated. She underwent 7 cycles of plasmapheresis (alternate days from 30th march to 12thapril).
On Day- 9 Her pupillary response to light and cough reflex was observed. Gradually improvement in her symptoms over the next 2 weeks.
On Day- 17 Mild motor activity of eyelid and foot movements, nodding of head, bilateral spontaneous lower limb movements, jaw & trunk movement.
Chair mobilization and physiotherapy was done. Next dose of IV IG was given, eyeball movement improved and initiated lip movements to communicate and gradually improved to being able to talk with speaking valve.
Her cough reflex and respiratory efforts improved and she was weaned off and decannulation done on May 30th. She was tolerating orals.
Condition at discharge:
- Sensory perception- intact
- Bowel and bladder sensation present
- Cranial nerve- intact
| RIGHT | LEFT | |
| POWER | ||
| Shoulder abduction/adduction/ | 3+/5 | 3+/5 |
| Shoulder flexion | 2/5 | 2/5 |
| Shoulder extension | 3+/5 | 3+/5 |
| Elbow flexion | 1/5 | 1/5 |
| Elbow extension | 3+/5 | 3+/5 |
| Wrist flexion/extension | 3/5 | 3/5 |
| Finger flexion/extension | 2/5 | 2/5 |
| Hip& knee flexion/extension | 4/5 | 4/5 |
| Ankle dorsiflexion and plantar flexion | 4/5 | 4/5 |
| REFLEXES | ||
| Biceps/triceps/supinator | mute | mute |
| Knee/ankle | mute | mute |
| plantar | flexor | mute |
She was discharged and transferred to rehabilitation centre .
DISCUSSION:
Miller Fisher Syndrome (MFS) is a rare, acquired nerve disease that is considered a variant of Guillain-Barré Syndrome (GBS). It typically presents with a triad of symptoms: ophthalmoplegia (paralysis or weakness of the eye muscles), ataxia (lack of voluntary coordination of muscle movements), and areflexia (absence of reflexes).
Ocular Movement showed total restriction on the both eyes
Key Features of Miller Fisher Syndrome:
Ophthalmoplegia: This involves weakness or paralysis of the muscles that control eye movements, leading to double vision and difficulty moving the eyes
Ataxia: Patients experience unsteady gait and coordination problems, making it difficult to walk or perform fine motor tasks
Areflexia: Reflexes, especially in the legs, are often absent or significantly diminished.
Pathophysiology:
MFS is believed to be an autoimmune disorder in which the body’s immune system mistakenly attacks its own nerves. The exact cause is unknown, but it is often preceded by a viral or bacterial infection. One common trigger is infection with Campylobacter jejuni, a bacterium that causes Gastroenteritis.
Diagnosis:
Clinical Examination: Identifying the classic triad of symptoms is the first step
Lumbar Puncture: This may show elevated protein levels in cerebrospinal fluid without a significant increase in white blood cells (albuminocytologic dissociation).
Nerve Conduction Studies: These can show signs of nerve dysfunction.
Anti-GQ1b Antibodies: The presence of these antibodies in the blood supports the diagnosis, as they are found in a significant number of MFS cases.
Treatment:
Intravenous Immunoglobulin (IVIG): Helps reduce the immune system’s attack on the nervous system.
Plasmapheresis (Plasma Exchange): This procedure removes antibodies from the blood.
Supportive Care: Managing symptoms such as pain, preventing complications, and providing physical therapy to assist with mobility and coordination.
Prognosis:
The prognosis for MFS is generally good. Most patients experience a significant recovery within weeks to months, although some may have lingering symptoms such as mild coordination issues or residual eye movement abnormalities.
MFS is a rare and complex condition, so specialized medical care is essential for proper diagnosis and treatment.
Case presentation done by Dr. Niveda R
Acknowledgement:
I would like to thank Dr.Aslesha Vijaay sheth (Head of Emergency Medicine Department)
Dr. Niveda R
Dept. of Emergency Medicine
Kauvery Hospital Chennai