Guillain-Barre syndrome presenting with severe hydrocephalus in a child.

Dear sir,

Guillain-Barre syndrome (GBS) is an acute polyneuropathy, presents itself with mainly progressive limb weakness, loss of deep tendon reflexes, mild sensory disturbance, and variable autonomic dysfunction.[1] GBS is rarely associated with hydrocephaly and papilledema.[23] In this case report, we describe the clinical course and neurophysiological and neuroimaging findings of a patient with GBS associated with hydrocephalus. The notable aspect of this case is that her hydrocephalus was treated medically without surgical intervention.

A 21-month-old girl was admitted in another hospital with progressive limb weakness, and had three episodes of seizures on 15th day of admission. Neuroimaging was performed which showed severe hydrocephaly [Figure 1a]. She was advised ventriculoperitoneal shunt insertion by surgeon, but parents refused any surgical intervention and she was referred to our hospital. She was tetraplegic and her neurologic examination showed dilated pupil with poor response to light, bilateral abducens palsy, papilledema in fundoscopy, absent gag reflex, and absent tendon reflex in all her limbs. She could feel painful stimulus with tearing and pursue objects by her eyes. Her electrodiagnostic studies including electromyography and nerve conduction velocity showed denervation signs including decreased action potential and absent F wave in motor nerves of limbs (peroneal, tibial, and median). She did not need mechanical ventilation during her illness. Electroencephalography was normal. She was diagnosed as GBS.

Figure 1

(a) Severe hydrocephaly at presentation (b) Hydrocephaly decreased without surgical intervention within 4 months (c) No hydrocephaly was seen 12 months later

We again recommended surgical intervention for her hydrocephalus, but parents refused it. We started supportive therapy and standard therapy for GBS including intravenous immunoglobulin 0.4 g/kg daily and methylprednisolone pulse 20 mg/kg/daily simultaneously for 5 days. Due to papilledema and hydrocephaly, her lumbar puncture was done after 1 month of treatment. Her cerebrospinal fluid (CSF) finding showed a high protein level 350 mg/dL without pleocytosis.

On 5th day of treatment, she could swallow by mouth with herself without using nasogastric tube. Her ability to move became better gradually and after 4 months she could walk without support and her funduscopy was normal and her brain image shows only ventricular dilatation without pressure marks [Figure 1b]. She was neurologically normal 12 months following the onset of GBS and her last brain image was nearly normal [Figure 1c].

GBS is regarded as a predominantly motor inflammatory polyneuropathy, with progressive weakness and areflexia.[1] GBS associated with central nervous system manifestations has been described earlier.[4]

Hydrocephalus associated with GBS is a rare but well-recognized complication. It can appear at any time in the course of the illness. The exact mechanism of development of hydrocephalus during GBS is unclear. It has been suggested that high concentration of CSF protein may lead to decreased CSF absorption in arachnoid villi. Other mechanisms such as increased osmotic pressure and cerebral edema are also hypothesized.[56]

Until now, few cases of GBS with hydrocephalus were reported.[3678] It is very rare and may be missed in GBS patients who have respiratory insufficiency and are ventilator dependent, due to their inability to communicate verbally. For this reason, some authors recommended neuroimaging in GBS patients with papilledema, headache, nausea, or vomiting, cognitive deficits, extremely high CSF protein concentration >300 mg/dL, high CSF pressure >200mm H2O or seizure.[46] In some cases acute hydrocephalus preceded the symptoms of GBS, so for this atypical presentation to avoid delaying appropriate treatment, GBS should be considered in the etiology of acute hydrocephalus especially when complicated with tetraparesis.[9]

In contrast to other reported cases who are older and can express headache and cognition more clearly, our patient was too young to do so. This case was one of the youngest cases who was affected with GBS and hydrocephaly, the youngest case reported earlier was a 1 month age patient who needed mechanical ventilation.[10]

Although surgical intervention has been approved for relieving increased intracranial pressure, our patient managed medically successfully. Recovery time in patient who were operated for ventriculoperitoneal shunt varied from 1 to 12 months and for those who were managed medically this period was 5-6 months.[6]

In our patient besides hydrocephalus-seizure episodes were also present. Although, seizures have been reported in GBS patients it is an uncommon presentation.

GBS in children may rarely present with severe hydrocephalus and regressing signs, its prognosis is usually good and it could be treated medically without CSF shunting. Monitoring with serial CT scan and fundoscopy should be especially recommended in young patients.