ECTRIMS eLearning

‘Sixteen syndrome’- Three Cases of Multiple Sclerosis Presenting with Pontine Lesions
ECTRIMS Learn. Hu M. 10/27/17; 200493; P838
Abstract

Abstract: P838

Type: Poster

Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis

Introduction: In Multiple Sclerosis (MS), complex cranial nerve palsies have been reported as a result of pontine demyelination. Pontine lesions can present with a variety of clinical presentations, depending on the structures involved. Presentations include internuclear opthalmoplegia,'one-and-a-half syndrome' and 'eight-and-a-half syndrome´.
Rarely reported in MS is the 'sixteen syndrome', in which patients exhibit bilateral complete horizontal gaze palsy associated with bilateral facial nerve weakness. Here the pontine lesion encompasses bilaterally the intrapontine facial nerve fascicles, sixth nerve nucleus, medial longitudinal fasciculus and pontine paramedian reticular formation.
Methods: We collected data on all cases of 'sixteen syndrome' presenting to a tertiary Neurology service in South West Wales.
Results: We present a case series of three patients presenting with 'sixteen syndrome' as a result of pontine demyelination, including eye movement videos and neuro-imaging.
The three patients presented to Morriston Hospital, Swansea within the last 12 months. In all cases there was a subacute history of dizziness and eye movement problems. The three patients exhibited bilateral complete horizontal gaze paralysis and bilateral facial nerve weakness, without evidence of other cranial nerve involvement.
Clinical findings were correlated with typical MRI features of pontine demyelination in all three cases, and they were all given a subsequent diagnosis of MS.
Conclusion: Although a rare entity, 'sixteen syndrome' should be recognised as a possible presenting sign of pontine demyelination. Prompt recognition of this sign allows clear anatomical localisation, and can facilitate early and appropriate diagnosis and treatment.
Disclosure:
M Hu: nothing to disclose.
W O Pickrell: nothing to disclose.
R Smith: nothing to disclose.
G Ingram: has received travel funding and/or speaker honoraria from Biogen, Roche, Merck Serono, Novartis, Teva, Genzyme Sanofi.
O R Pearson: served on the scientific advisory board for Biogen, Novartis, Roche, UK MS Register and has received travel funding and/or speaker honoraria from Biogen, Roche, Merck Serono, Novartis, Teva, Genzyme Sanofi.

Abstract: P838

Type: Poster

Abstract Category: Clinical aspects of MS - 1 Diagnosis and differential diagnosis

Introduction: In Multiple Sclerosis (MS), complex cranial nerve palsies have been reported as a result of pontine demyelination. Pontine lesions can present with a variety of clinical presentations, depending on the structures involved. Presentations include internuclear opthalmoplegia,'one-and-a-half syndrome' and 'eight-and-a-half syndrome´.
Rarely reported in MS is the 'sixteen syndrome', in which patients exhibit bilateral complete horizontal gaze palsy associated with bilateral facial nerve weakness. Here the pontine lesion encompasses bilaterally the intrapontine facial nerve fascicles, sixth nerve nucleus, medial longitudinal fasciculus and pontine paramedian reticular formation.
Methods: We collected data on all cases of 'sixteen syndrome' presenting to a tertiary Neurology service in South West Wales.
Results: We present a case series of three patients presenting with 'sixteen syndrome' as a result of pontine demyelination, including eye movement videos and neuro-imaging.
The three patients presented to Morriston Hospital, Swansea within the last 12 months. In all cases there was a subacute history of dizziness and eye movement problems. The three patients exhibited bilateral complete horizontal gaze paralysis and bilateral facial nerve weakness, without evidence of other cranial nerve involvement.
Clinical findings were correlated with typical MRI features of pontine demyelination in all three cases, and they were all given a subsequent diagnosis of MS.
Conclusion: Although a rare entity, 'sixteen syndrome' should be recognised as a possible presenting sign of pontine demyelination. Prompt recognition of this sign allows clear anatomical localisation, and can facilitate early and appropriate diagnosis and treatment.
Disclosure:
M Hu: nothing to disclose.
W O Pickrell: nothing to disclose.
R Smith: nothing to disclose.
G Ingram: has received travel funding and/or speaker honoraria from Biogen, Roche, Merck Serono, Novartis, Teva, Genzyme Sanofi.
O R Pearson: served on the scientific advisory board for Biogen, Novartis, Roche, UK MS Register and has received travel funding and/or speaker honoraria from Biogen, Roche, Merck Serono, Novartis, Teva, Genzyme Sanofi.

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