| Sub-title | |
| Authors | V Said Conti S Attard Montalto |
| Abstract | Introduction: Guillain-Barre syndrome (GBS) results in post-infectious demyelination that causes motor but sometimes also sensory polyneuropathy. It usually follows 10 days after a non-specific viral infection. Weakness usually begins in the lower extremities and progressively involves the trunk, upper limbs and bulbar muscles over days or weeks. Bulbar involvement occurs in about 50% of cases. The most common cranial nerve involved is the facial nerve. Miller-Fisher syndrome (MFS) is a variant of GBS involving the triad ataxia, ophthalmoplegia and areflexia. The pathophysiology of the syndrome can be explained through GQ1b ganglioside autoantibodies seen in 96% of cases, which recognize epitopes expressed specifically in the nodal regions of oculomotor nerves and cerebellar neurons. Organisms such as Mycoplasma pneumoniae contain cell walls that are capable of inducing the production of autoantibodies. We present the case of a child who presented with ataxia and ophthalmoplegia but in whom the reflexes were present throughout the illness. Case report: A 9 year old boy presented with a 3 day history of slurred speech and a 1 day history of bilateral lower limb weakness, dizziness and blurred vision. He had complained of a sore throat one week previously. On examination he had neck and truncal weakness and reduced power in all muscle groups of both upper and lower limbs (MRC grade 4) with normal tone and reflexes; there were no cerebellar signs and the plantar reflexes were downgoing. Sensation was not impaired. He had dysarthria, dysphonia and dysphagia. On the second day after presentation he developed ptosis and a squint and his gait became increasingly unsteady. CSF protein levels and cytology were normal. He was started on intravenous immunoglobulin, which was continued for 5 days. His weakness became progressively worse (MRC grade 3), with increasing ptosis and ophthalmoplegia but with preservation of all limb reflexes. Repeat examination of the CSF showed a pleocytosis but normal protein levels. He showed clinical signs of improvement by the end of the second week. CSF examination at this stage was normal. He continued improving steadily and was discharged after 1 month from presentation. 3 months later he still had minimal facial nerve weakness but with completely normal power in his limbs and trunk. Other investigations included: normal CT and MRI brain scans; negative serology for herpes virus and CMV; negative CSF for herpes zoster, mycoplasma and acid-fast bacilli; negative blood and CSF cultures. Serum Mycoplasma IgM was positive on day 1 and negative on day 14 when Mycoplasma IgG became positive. GQ1b ganglioside autoantibodies IgG and IgM were negative. Conclusion: This patient presented with signs suggestive of MFS associated with mycoplasma infection, but in whom the deep tendon reflexes remained preserved. In this regard, this case was a variant of the Miller Fisher variant of GBS. |
Published in: | |
| Journal | Malta Medical Journal |
| Volume | Volume 15 (suppl) |
| Pages | - |
| Date | |
| Link to journal | |
| Key words | mycoplasma-related, Miller-Fisher variant, Guillain-Barre syndrome, case report |