MILLER FISHER VARIANT OF GUILLAIN-BARRÉ SYNDROME ON A CHILD: CASE REPORT

LAÍS DE OLIVEIRA LIMA, MICHAELA DE OLIVEIRA TOGNINI, TAISE NAMIE NAKATA

Resumo


Introduction: Guillain-Barré syndrome is an acute polyneuropathy characterized by peripheral nerve
inflammation and demyelination, probably secondary to an immune response against myelin antigens.
GBS is clinically diagnosed and supported by complementary exams (cerebrospinal fluid investigation and
eletroneuromyography). It is a monophasic disease that rarely presents relapse and is characterized by
rapid, progressive and ascendant muscle weakness, associated with a protein-cytological dissociation of
the cerebrospinal fluid. In the present work, we describe a case report of Miller Fisher syndrome, a rare
subtype of GBS characterized by the clinical triad: ophthalmoplegia, ataxia and areflexia. Case report:
M.F.Z. patient, female, 5 years old, from and residing in Campo Grande, MS. Father reported that child
began presenting flu-like symptoms three weeks ago, lasting for three days and symptomatic
improvement after use of medication. Two weeks ago, she started with right eyelid ptosis, being referred
to evaluation with a neurologist at the Santa Casa which presented unaltered CT scan results. Patient
worsened after three days with decreased strength in the lower limbs and upper limbs and difficulty
walking. On examination M.F.Z. was conscious without sensory changes, distal paresis in upper and
steeper in the lower limbs with deep areflexia and right eyelid ptosis. Father denied previous diseases of
the child and family. Gestational maternal history and birth without complications. After 10 days of
evolution, elevated cerebrospinal fluid protein (110.70 mg/dL) was found and the child was diagnosed
with Miller Fisher syndrome after the exclusion of other conditions.The underlying disease of the patient
was treated with IV Ig administered at a dose of 2 g/kg, and 0.5/kg/day for 4 days. The patient showed
regression of motor deficit, though still with a slight ptosis at hospital discharge. Discussion: Miller Fisher
syndrome is extremely rare in children and is a diagnostic challenge at those ages. It presents bimodal
trend with a peak of involvement between the 2nd and 3rd decade and another between the 5th and 7th
decade of life. However, 20% of all cases occur in children under the age of 10 years. Miller Fish syndrome
may be associated with infectious, autoimmune and neoplastic diseases. It was included as GBS subtype
due to the presence of albumino-cytologic dissociation and spontaneous recovery. Despite the weakness
not being the main feature, patients have moderate weakness of limbs and decreased proprioception,
without sensory loss. The diagnosis is supported by the clinical manifestations and electrophysiological
and cerebrospinal fluid analysis tests. The test can have unaltered results during the first week of the
disease. Typical changes are found from the tenth day of evolution. There are two forms of treatment:
plasmapheresis and intravenous human immunoglobulin, which acts by modulating the immune system.
International results of randomized trials demonstrated equivalent efficacy of both these treatments. The
outcome is usually good. After reaching the summit, muscle strength returns first proximally then distally,
and may have improved motor deficits within 6 months after the end of treatment. Approximately 4 to
15% of patients die and 20% are left with some residual damage, even when treated.

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