Neuropediatrics 2018; 49(S 02): S1-S69
DOI: 10.1055/s-0038-1675980
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Georg Thieme Verlag KG Stuttgart · New York

P 1166. Isolated Oculomotor Nerve Palsy as Primary Manifestation of Guillain–Barré’s Syndrome—A Case Report with Focus on Clinical and Electrophysiological Features

Ursula von Mengershausen
1  Ostschweizer Kinderspital, Zentrum für Kinderneurologie, Entwicklung und Rehabilitation, St. Gallen, Switzerland
,
Philip Broser
1  Ostschweizer Kinderspital, Zentrum für Kinderneurologie, Entwicklung und Rehabilitation, St. Gallen, Switzerland
,
Oliver Maier
1  Ostschweizer Kinderspital, Zentrum für Kinderneurologie, Entwicklung und Rehabilitation, St. Gallen, Switzerland
› Author Affiliations
Further Information

Publication History

Publication Date:
30 October 2018 (online)

 

Background: Guillain–Barré’s syndrome (GBS) in childhood is a postinfectious multifocal demyelination and/or axonal damage in the spinal cord and peripheral nerves. The disease cause is assumed to be an autoimmunological process. The onset is typically acute or subacute with symmetrically ascending paralysis with reduced or absent tendon reflexes. Some patients show involvement of the cranial nerves and vegetative symptoms. In severe cases, the ascending paralysis leads to respiratory insufficiency. A rare variant of GBS is Miller–Fisher’s syndrome (MFS), characterized by the clinical trial of cranial nerve palsy, ataxia, and areflexia.

Method/Results: We report a 4-year-old girl presenting with an isolated oculomotor nerve palsy as initial symptom, in the further course, she developed the typical symptoms of GBS. The girl presented with an acute isolated oculomotor palsy on the right more than on the left side. At this time, she recovered from a varicella infection. There were no other neurological abnormalities in the clinical examination, in particular, no ataxia, no limb weakness, and no hypo- or areflexia. In the craniocerebral magnetic resonance imaging (MRI), an isolated neuritis of the oculomotor nerves was present with an otherwise unremarkable intracranial finding. With suspicion of an isolated cranial nerve palsy, we administered intravenous steroid for 3 days. Oculomotor nerve palsy did not improve and the girl became more and more irritable, refused walking, and on day 5, she developed a paralysis of the lower limbs with areflexia. Cerebrospinal fluid showed no albuminocytological dissociation. Nerve conduction studies (tibial nerve) showed a nontriggerable H-reflex and absent F-waves. The electrophysiological findings and the current clinical presentation were consistent with an acute disorder of the anterior motor neurons. Spinal MRI showed contrast enhancement along the roots consistent with polyradiculitis. The specific ganglioside antibodies were all negative. The girl was then treated with intravenous immunoglobulins over 5 days. Clinically, there was little improvement over about a week (“plateau phase”), in the further course, the patient recovered continuously and was again able to stand with support on day 19. The oculomotor nerve palsy improved as well. The girl was then referred to a rehabilitation hospital to continue rehabilitative treatment.

Conclusion: Our case report shows that isolated cranial nerve palsy—in our case isolated oculomotor nerve palsy—might be the first symptom present in GBS. The girl had no ataxia, hypo-, or areflexia at the time of initial presentation. Only later, she developed typical symptoms of GBS with areflexia and lower limb paralysis (day 5). We interpret the clinical course as a GBS spectrum variant.