Multiple Sclerosis and Related Disorders (2014) 3, 398–401
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/msard
CASE REPORT
Parinaud's syndrome – A rare presentation of clinically isolated syndrome S. Gnanapavana,n, E. Sillerya, J.F. Achesonb, A.T. Toosyc a
Department of Neuroimmunology, UCL Institute of Neurology, and National Hospital for Neurology and Neurosurgery, Queen Square, WC1N 3BG, UK b Department of Neuro-ophthalmology, National Hospital for Neurology and Neurosurgery, Queen Square, WC1N 3BG, UK c Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, University College London, WC1N 3BG, UK Received 27 June 2013; received in revised form 3 September 2013; accepted 6 September 2013
1.
KEYWORDS
Abstract
Parinaud's syndrome; Clinically isolated syndrome; Pre-tectal lesion; Demyelination; Convergence–retraction nystagmus; Vertical diplopia
We present a 26 year old Pakistani lady with first presentation of a demyelinating event, presenting as Parinaud's syndrome. The video demonstrates a convergence–retraction nystagmus on upgaze and failure of accommodation, and her brain imaging confirms a corresponding pre-tectal contrast enhancing T2 hyperintense lesion suggestive of demyelination. A review of the literature is presented. & 2013 Elsevier B.V. All rights reserved.
Introduction
Parinaud's syndrome or dorsal mid brain syndrome was first described by Parinaud (1883) with vertical paralysis and/or convergence paralysis and includes a putative case of multiple sclerosis (MS). The case descriptions of the syndrome have since widened to include convergence–retraction nystagmus, pathologic eyelid retraction (Collier's sign) and Pseudo-Argyll-Robertson pupils (mid-dilated pupils with lightnear dissociation). The differential diagnosis includes pineal gland tumours, MS, acute hydrocephalus, angiomas, infections n
Corresponding author. Tel.: +44 7930 660 210. E-mail address: [email protected] (S. Gnanapavan).
2211-0348/$ - see front matter & 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.msard.2013.09.003
(e.g. toxoplasmosis), strokes (e.g. thalamic stroke or haemorrhage), and rarely tonic–clonic seizure (Clarke et al. 2009).
1.1.
Clinical history
A 26 year old previously well Pakistani lady who moved to the UK at the age of 11 presented with a 1 week history of left sided headache and sharp intermittent pains in the occiput. This would progressively worsen through the day and in bright lights. As the headaches became severe, she developed double vision upon looking up and down, associated with nausea. There was no family history of note, or of consanguinity. She had not previously experienced oral or genital ulceration. On examination her visual acuity, colour
Parinaud's syndrome vision, funduscopy, pupillary light reflexes and visual fields were normal. Examination of ocular motility revealed some upgaze limitation, a mild left over right 3 dioptre vertical concomitant tropia (Fig. 1), convergence retraction nystagmus on elevation (video) and convergence insufficiency (video). Supplementary material related to this article can be found online at http://dx.doi.org/10.1016/j.msard.2013.09.003 CT head scan demonstrated an enlarged lesion in the region of the pineal gland which was bright on post-contrast (not shown) and a subsequent magnetic resonance imaging (MRI) study (Fig. 2) revealed a T2 hyperintense lesion
Fig. 1
399 superiorly on the left side of the mesencephephalon in the periaqueductal region extending into the subthalamic region. An inflammatory panel revealed a mildly elevated erythrocyte sedimentation rate (ESR) at 32, but a normal autoantibody screen, serum protein electrophoresis, vitamin B12/B1, folate, angiotensin converting enzyme(ACE), thyroid function, Human immunodeficiency virus (HIV), toxoplasma, quantiferon Tuberculosis (TB) Gold, neuronal antibodies, human chorionic gonadotropin (HCG) and lactate dehydrogenase (LDH). CSF examination was normal aside from a WCC 6 (lymphocytes) per mm3 and positive oligoclonal bands in the CSF only.
The Hess chart demonstrates a mild left over right tropia responsible for the patient's vertical diplopia.
400
S. Gnanapavan et al.
COR T2 Flair SAG T2 FSE
AX T1 FSE + C Fig. 2 MRI brain at presentation: there is a small well circumscribed T2 hyperintense lesion superiorly in the left side of the mesencephalon in the periaqueductal region (COR T2 Flair=Coronal T2 Flair). The lesion does show faint peripheral enhancement (AX T1 FSE+C=Axial fat suppressed T1 post-contrast). The left superior periaqueductal lesion does therefore appear to be a solitary intraparenchymal lesion and an inflammatory lesion would seen more likely than an ischaemic one (SAG T2 FSE=Sagittal T2 fat suppressed). T2 COR Flair
T2 COR Flair
Fig. 3 Follow-up MRI Brain 3 months later: The small left-sided T2- and FLAIR-hyperintense peri-aqueductal midbrain lesion appears smaller than on the previous. A new small focus of abnormal signal is present within the left cingulate juxtacortical white matter (arrow).
Her eye symptoms improved over the course of the next few weeks without treatment. A follow up MRI study 2 months later demonstrated a reduction in the size of the peri-aqueductal lesion with no discernible enhancement (Fig. 3). A new nonenhancing lesion was also present in the left cingulate juxtacortical white matter. The combination of findings was most in keeping with an inflammatory demyelinating pathology.
2.
Discussion
Complex eye movement disorders in the context of MS are not unusual and commonly involve internuclear opthalmoplegias, cerebellar eye movement disorders and pendular nystagmus
(Barnes and McDonald, 1992). Parinaud's syndrome on the other hand is an unusual primary manifestation of demyelination (clinically isolated syndrome) with only a handful of cases described (Slyman and Kline, 1981; Quint et al., 1993; Lee et al., 2003; Moreira et al., 2012). The phenomenon should therefore always be a diagnosis of exclusion in demyelination. A diagnosis of MS based on the revised McDonald diagnostic criteria (2010) was only possible after an interval MRI, which demonstrated both dissemination by space and time. The supranuclear vertical gaze palsy is characteristic in Parinaud's and results from involvement of the rostral interstitial nuclei of the medial longitudinal fasciculus (riMLF), its excitatory efferents projecting to the oculomotor nuclei, or their afferent pathways (Pierrot-Deseilligny et al., 1982).
Parinaud's syndrome Attempts at upward gaze produce convergence and globe retraction. Skew deviation may also be present, as demonstrated in our patient, resulting in vertical diplopia. Unilateral midbrain lesions can create the same ocular motor syndrome by interrupting both projections through the posterior commissure (Auerbach et al., 1982; Ranalli et al., 1988; Smith and Laguna, 1981). Treatment is dependent on the underlying aetiology, but in our case, symptoms resolved spontaneously over a period of a few weeks without the need for corticosteroids.
Conflict of interest Authors report no conflicts of interest.
References Auerbach SH, De Piero TJ, Romanul F. Sylvian aqueduct syndrome caused by unilateral midbrain lesion. Annals of Neurology 1982;11:91–4. Barnes D, McDonald WI. The ocular manifestations of multiple sclerosis. Journal of Neurology, Neurosurgery and Psychiatry 1992;55:863–8.
401 Clarke C, Howard R, Rosser M, Shorvon S. Queen Square Textbook of Neurology. London, 2009, UK: Wiley-Blackwell. Lee WB, Berger JR, Lexington KY, O'Halloran HS. Parinaud syndrome heralding MS. Neurology 2003;60:322. Moreira IP, Carneiro A, Santos E. Parinaud syndrome: a rare relapse in multiple sclerosis. Neuro-Opthalmology 2012;36:133–4. Parinaud H. Paralysie des mouvements associés des yeux. Archives de Neurologie, Paris 1883;5:145–72. Pierrot-Deseilligny C, Chain F, Gray M, et al. Parinaud's syndrome. Brain 1982;105:667–96. Quint DJ, Cornblath WT, Trobe JD. Multiple sclerosis presenting as Parinaud syndrome. AJNR American Journal of Neuroradiology 1993;14:1200–2. Ranalli PJ, Sharpe JA, Fletcher WA. Palsy of upward and downward saccadic, pursuit, and vestibular movements with a unilateral midbrain lesion: pathophysiological correlations. Neurology 1988;38:114–22. Slyman JF, Kline LB. Dorsal midbrain syndrome in multiple sclerosis. Neurology 1981;31:196–8. Smith MS, Laguna JF. Upward gaze paralysis following unilateral pretectal infarction. Computerized tomography correlation. Archives of Neurology 1981;38:127–9.
Available online at www.sciencedirect.com
journal homepage: www.elsevier.com/locate/msard
CASE REPORT
Parinaud's syndrome – A rare presentation of clinically isolated syndrome S. Gnanapavana,n, E. Sillerya, J.F. Achesonb, A.T. Toosyc a
Department of Neuroimmunology, UCL Institute of Neurology, and National Hospital for Neurology and Neurosurgery, Queen Square, WC1N 3BG, UK b Department of Neuro-ophthalmology, National Hospital for Neurology and Neurosurgery, Queen Square, WC1N 3BG, UK c Department of Brain Repair and Rehabilitation, UCL Institute of Neurology, University College London, WC1N 3BG, UK Received 27 June 2013; received in revised form 3 September 2013; accepted 6 September 2013
1.
KEYWORDS
Abstract
Parinaud's syndrome; Clinically isolated syndrome; Pre-tectal lesion; Demyelination; Convergence–retraction nystagmus; Vertical diplopia
We present a 26 year old Pakistani lady with first presentation of a demyelinating event, presenting as Parinaud's syndrome. The video demonstrates a convergence–retraction nystagmus on upgaze and failure of accommodation, and her brain imaging confirms a corresponding pre-tectal contrast enhancing T2 hyperintense lesion suggestive of demyelination. A review of the literature is presented. & 2013 Elsevier B.V. All rights reserved.
Introduction
Parinaud's syndrome or dorsal mid brain syndrome was first described by Parinaud (1883) with vertical paralysis and/or convergence paralysis and includes a putative case of multiple sclerosis (MS). The case descriptions of the syndrome have since widened to include convergence–retraction nystagmus, pathologic eyelid retraction (Collier's sign) and Pseudo-Argyll-Robertson pupils (mid-dilated pupils with lightnear dissociation). The differential diagnosis includes pineal gland tumours, MS, acute hydrocephalus, angiomas, infections n
Corresponding author. Tel.: +44 7930 660 210. E-mail address: [email protected] (S. Gnanapavan).
2211-0348/$ - see front matter & 2013 Elsevier B.V. All rights reserved. http://dx.doi.org/10.1016/j.msard.2013.09.003
(e.g. toxoplasmosis), strokes (e.g. thalamic stroke or haemorrhage), and rarely tonic–clonic seizure (Clarke et al. 2009).
1.1.
Clinical history
A 26 year old previously well Pakistani lady who moved to the UK at the age of 11 presented with a 1 week history of left sided headache and sharp intermittent pains in the occiput. This would progressively worsen through the day and in bright lights. As the headaches became severe, she developed double vision upon looking up and down, associated with nausea. There was no family history of note, or of consanguinity. She had not previously experienced oral or genital ulceration. On examination her visual acuity, colour
Parinaud's syndrome vision, funduscopy, pupillary light reflexes and visual fields were normal. Examination of ocular motility revealed some upgaze limitation, a mild left over right 3 dioptre vertical concomitant tropia (Fig. 1), convergence retraction nystagmus on elevation (video) and convergence insufficiency (video). Supplementary material related to this article can be found online at http://dx.doi.org/10.1016/j.msard.2013.09.003 CT head scan demonstrated an enlarged lesion in the region of the pineal gland which was bright on post-contrast (not shown) and a subsequent magnetic resonance imaging (MRI) study (Fig. 2) revealed a T2 hyperintense lesion
Fig. 1
399 superiorly on the left side of the mesencephephalon in the periaqueductal region extending into the subthalamic region. An inflammatory panel revealed a mildly elevated erythrocyte sedimentation rate (ESR) at 32, but a normal autoantibody screen, serum protein electrophoresis, vitamin B12/B1, folate, angiotensin converting enzyme(ACE), thyroid function, Human immunodeficiency virus (HIV), toxoplasma, quantiferon Tuberculosis (TB) Gold, neuronal antibodies, human chorionic gonadotropin (HCG) and lactate dehydrogenase (LDH). CSF examination was normal aside from a WCC 6 (lymphocytes) per mm3 and positive oligoclonal bands in the CSF only.
The Hess chart demonstrates a mild left over right tropia responsible for the patient's vertical diplopia.
400
S. Gnanapavan et al.
COR T2 Flair SAG T2 FSE
AX T1 FSE + C Fig. 2 MRI brain at presentation: there is a small well circumscribed T2 hyperintense lesion superiorly in the left side of the mesencephalon in the periaqueductal region (COR T2 Flair=Coronal T2 Flair). The lesion does show faint peripheral enhancement (AX T1 FSE+C=Axial fat suppressed T1 post-contrast). The left superior periaqueductal lesion does therefore appear to be a solitary intraparenchymal lesion and an inflammatory lesion would seen more likely than an ischaemic one (SAG T2 FSE=Sagittal T2 fat suppressed). T2 COR Flair
T2 COR Flair
Fig. 3 Follow-up MRI Brain 3 months later: The small left-sided T2- and FLAIR-hyperintense peri-aqueductal midbrain lesion appears smaller than on the previous. A new small focus of abnormal signal is present within the left cingulate juxtacortical white matter (arrow).
Her eye symptoms improved over the course of the next few weeks without treatment. A follow up MRI study 2 months later demonstrated a reduction in the size of the peri-aqueductal lesion with no discernible enhancement (Fig. 3). A new nonenhancing lesion was also present in the left cingulate juxtacortical white matter. The combination of findings was most in keeping with an inflammatory demyelinating pathology.
2.
Discussion
Complex eye movement disorders in the context of MS are not unusual and commonly involve internuclear opthalmoplegias, cerebellar eye movement disorders and pendular nystagmus
(Barnes and McDonald, 1992). Parinaud's syndrome on the other hand is an unusual primary manifestation of demyelination (clinically isolated syndrome) with only a handful of cases described (Slyman and Kline, 1981; Quint et al., 1993; Lee et al., 2003; Moreira et al., 2012). The phenomenon should therefore always be a diagnosis of exclusion in demyelination. A diagnosis of MS based on the revised McDonald diagnostic criteria (2010) was only possible after an interval MRI, which demonstrated both dissemination by space and time. The supranuclear vertical gaze palsy is characteristic in Parinaud's and results from involvement of the rostral interstitial nuclei of the medial longitudinal fasciculus (riMLF), its excitatory efferents projecting to the oculomotor nuclei, or their afferent pathways (Pierrot-Deseilligny et al., 1982).
Parinaud's syndrome Attempts at upward gaze produce convergence and globe retraction. Skew deviation may also be present, as demonstrated in our patient, resulting in vertical diplopia. Unilateral midbrain lesions can create the same ocular motor syndrome by interrupting both projections through the posterior commissure (Auerbach et al., 1982; Ranalli et al., 1988; Smith and Laguna, 1981). Treatment is dependent on the underlying aetiology, but in our case, symptoms resolved spontaneously over a period of a few weeks without the need for corticosteroids.
Conflict of interest Authors report no conflicts of interest.
References Auerbach SH, De Piero TJ, Romanul F. Sylvian aqueduct syndrome caused by unilateral midbrain lesion. Annals of Neurology 1982;11:91–4. Barnes D, McDonald WI. The ocular manifestations of multiple sclerosis. Journal of Neurology, Neurosurgery and Psychiatry 1992;55:863–8.
401 Clarke C, Howard R, Rosser M, Shorvon S. Queen Square Textbook of Neurology. London, 2009, UK: Wiley-Blackwell. Lee WB, Berger JR, Lexington KY, O'Halloran HS. Parinaud syndrome heralding MS. Neurology 2003;60:322. Moreira IP, Carneiro A, Santos E. Parinaud syndrome: a rare relapse in multiple sclerosis. Neuro-Opthalmology 2012;36:133–4. Parinaud H. Paralysie des mouvements associés des yeux. Archives de Neurologie, Paris 1883;5:145–72. Pierrot-Deseilligny C, Chain F, Gray M, et al. Parinaud's syndrome. Brain 1982;105:667–96. Quint DJ, Cornblath WT, Trobe JD. Multiple sclerosis presenting as Parinaud syndrome. AJNR American Journal of Neuroradiology 1993;14:1200–2. Ranalli PJ, Sharpe JA, Fletcher WA. Palsy of upward and downward saccadic, pursuit, and vestibular movements with a unilateral midbrain lesion: pathophysiological correlations. Neurology 1988;38:114–22. Slyman JF, Kline LB. Dorsal midbrain syndrome in multiple sclerosis. Neurology 1981;31:196–8. Smith MS, Laguna JF. Upward gaze paralysis following unilateral pretectal infarction. Computerized tomography correlation. Archives of Neurology 1981;38:127–9.
