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Editor,—We report a child with rapidly progressive and severe acute Guillain-Barré syndrome (GBS) with complete ophthalmoplegia (ptosis, loss of eye movements, and pupillary paresis).
A previously well 13 year old boy presented with a seven hour history of increasing breathlessness, aching of his facial and neck muscles, and a rapidly progressive weakness of all four limbs. The patient had complained of a mild coryzal illness for the previous five days. The patient was afebrile and there was no evidence of rash, tick bite, or lymphadenopathy. He was ‘alert’ and his breathing pattern was shallow. Neurological examination demonstrated marked hypotonia and diffuse muscle weakness (Medical Research Council (MRC) classification grade 2); muscle stretch and abdominal reflexes were absent. Plantar responses were flexor. Extraocular eye movements were absent; there was some movement of the eyelids (MRC grade 1–2) and pupillary responses to accommodation and light (direct and consensual) were normal. Because of significant hypoventilation, the patient required immediate intubation and ventilatory support. Three doses of intravenous immunoglobulin at 1 g/kg/dose were administered over three consecutive days.
By the third day of admission paralysis was total, with no movement seen in any muscle. Autonomic dysfunction was evident from day one through day 15 of admission, manifested by labile blood pressure, a sinus tachycardia, sweating and flushing, urinary incontinence, and constipation. From the third day of admission the patient’s pupils were reacting sluggishly to light; by the fourth day the pupils were 4 mm in diameter and unreactive to light and accommodation. Resolution of the iridoplegia began on the 11th day of admission, initially to light and subsequently to accommodation; the pupils had returned to normal (size and reactivity) by day 15.
Recovery from paralysis began on the 14th day in a cephalocaudal progression, beginning with blinking of the eyelids and lateral movements of the head. The patient remained ventilator dependent for 59 days. Ten months after admission, the patient is able to walk unaided. The muscle power in the upper limbs (proximally and distally) is grade 4, and in the proximal and distal lower limbs, grade 4 and 2 respectively.
Magnetic resonance imaging of the head and spine were normal. Protein concentrations in the cerebrospinal fluid were 0.54 and 4.46 g/l on the first and seventh days of admission respectively (upper limit of normal 0.45 g/l); the rest of the cerebrospinal fluid biochemistry and white cell counts were normal. Urine porphyrins were negative. Bacterial and viral culture and viral antibody titres were negative. Electrophysiology, undertaken on day 40 of admission, demonstrated absent sensory action potentials and a markedly delayed M response from the right biceps, consistent with a demyelinating motor (and sensory) neuropathy with severe secondary axonal degeneration.
The clinical features and investigations in this patient were entirely consistent with a diagnosis of acute GBS, and diphtheria or a brainstem lesion were excluded. The progression was extremely rapid and the patient’s recovery has been gradual but sustained, and is not yet complete. Pupillary involvement in GBS appears to be rare as it is not cited in at least one reference text1; furthermore, it has been stated that ocular pupillary abnormalities do not occur in GBS.2 However, pupillary abnormalities have been reported in seven adults with GBS; all except one required ventilatory support and two (the non-ventilated, and one of the ventilated patients) died of a cardiac arrhythmia.3-8 Although there may be a reporting bias, pupillary involvement would appear to be associated with severe disease and an increased risk of dying. The pathophysiology is unclear but almost certainly represents a manifestation of autonomic dysfunction. Pharmacological testing previously has suggested either simultaneous postganglionic involvement of sympathetic and parasympathetic nerves or isolated parasympathetic involvement.4 In our patient, pupillary paresis developed at the height of autonomic instability. It appeared to affect both sympathetic and parasympathetic nerves, although this was not confirmed pharmacologically. Bilateral ptosis and failure of full pupillary dilatation in the dark indicated sympathetic involvement, while absence of light and consensual responses suggested parasympathetic involvement. The ‘mid-point’ dilatation of the pupils was probably due to the presence of intrinsic muscle tone within the pupillary muscles. We believe that the recognition of iridoplegia as an associated feature of GBS is important and its presence warrants intensive monitoring.