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Título

MILLER FISHER SYNDROME WITH IDIOPATHIC INTRACRANIAL HYPERTENSION: A CASE REPORT

Apresentação do caso

A 11-years-old girl, previously healthy, presented with a respiratory infection. Few weeks later, developed myalgia and proximal weakness. Her symptoms worsened promptly and, in a week, she lost the ability to walk. She was admitted to our hospital presenting confusion, ataxia, dysmetria, facial paralysis, nuchal rigidity, gaze palsy and hyporeflexia. Cerebrospinal fluid (CSF) showed albuminocytological dissociation and an opening pressure of 330 mmH20 with a normal brain MRI and fundus examination. Electromyoneurography indicated a recent sensorimotor axonal polyradiculoneuropathy. Based on these clinical and neurophysiological data, the diagnosis of Miller Fisher syndrome (MFS) was established and she received intravenous immunoglobulin for 4 days. Two weeks later, she complained of visual acuity worsening and bilateral optic disc swelling was noticed. A new brain and orbital MRI showed dilation of both optic nerve sheath and flattening of the posterior sclera. An idiopathic intracranial hypertension (IIH) was diagnosed and acetazolamide started, followed by a significant clinical improvement.

Discussão

MFS is an acute demyelinating disease of the peripheral nervous system. It is considered a variant of Guillain–Barré syndrome (GBS), and is characterized by: ophthalmoplegia, ataxia and hyporeflexia. IIH is rare among patients with GBS. Among children with GBS in a pediatric survey, only 4% presented with papilledema, usually developing days to weeks after the onset of symptoms. IIH is characterized by raised intracranial pressure without an obvious cerebral pathology. CSF is normal and shows a raised opening pressure. The explanation for the occurrence of both syndromes is not yet explained, but it might be caused by high CSF protein in GBS, that blocks CSF path at the arachnoid granulations.

Comentários finais

Although rare, raised intracranial pressure (with or without papilledema), might be a feature of GBS and its variants. Early diagnosis of IIH in these cases is important, since it allows symptomatic management and can prevent permanent visual loss associated with papilledema. Therefore, we propose that every patient with GBS suspected should have the CSF opening pressure monitored in every lumbar puncture performed. The timing of fundus examination is also important, since papilledema may be missed if examination is performed early in the course of the disease.

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