Case report of a patient with ‘one-and-a-half plus syndrome: nine syndrome’

Case report of a patient with ‘one-and-a-half plus syndrome: nine syndrome’ This case talks about ‘One-and-a-half plus syndrome’, a clinical syndrome affecting binocular vision and facial nerve. One-and-a- half plus syndrome is a less known clinical syndrome which constitutes of a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other direction. Despite the known association between ischemia, autoimmune disorders, multiple sclerosis, with mono neuritis multiplex resulting in extra ocular movement disorder, one-and-a-half plus syndrome is rarely considered in the differential diagnosis of eye ball movement disorders, as many clinicians are not able to diagnose such a case as ‘ the eyes don’tsee what theminddoesn’tknow’. Our report aims to raise awareness about connective tissue disorders presenting as neuro-ophthalmological syndrome, as early recognition can accelerate diagnosis and decrease the morbidity. had a ‘static left eye’ The family gave history of intermittent INTRODUCTION high grade fever, with profuse vomiting. The one-and-a half syndrome (1 and ½, OAHS) is a neurological On examination, patient was restless and irritable: she had disorder resulting in disorganization of conjugate eye movement: blood pressure of 110/60 mmHg, with no postural drop; pulse exotropia, conjugate horizontal gaze palsy (CHGP) in one direc- 90 b/min, respiratory rate 22 b/min and temperature 37.8°C. Oral tion and internuclear ophthalmoplegia (INO) in the other [1–5]. ulcers were present. During neurological examination she had Conjugate Horizontal gaze palsy (CHGP) and internuclear right side hemiataxia although there was not any focal motor def- opthalmoplegia/nystagmus is present if there is extensive icit. Cerebellar examination was unremarkable. Gag reflex present lesion involving the centers and tracts (medial longitudinal fas- on both sides. Brudzinski’s sign was positive and cranial nerve iculus) controlling binocular vision. examination revealed that the left eye could not move superiorly, The lesion may be infarction, atherosclerosis, hemorrhage inferiorly, medially nor laterally and there was right eye lateral in the basilar artery territory, demyelination, tuberculoma or gaze nystagmus. Left side facial palsy was also present. any other SOL. A clinical diagnosis of ‘One-and-a-half syndrome along with left side facial palsy’ was made and keeping in view of the pos- sible etiologies, she was planned to be worked up extensively, i.e CASE DESCRIPTION on lines of viral, bacterial, infectious and autoimmune etiologies. Nonspecific routine Laboratory tests revealed: hemoglobin A 16-year-old female was referred from a tertiary care hospital for steady gait, tendency to fall on right side,vertigo right sided 9.1 mg/dl, low HCT 31%, MCV 64 fl, MCHC 39 g/dl, MCH 19 pg, ESR 32 mm/h, platelets 331 × 10 /l, WBC 7.6. facial deviation, disorientation to persons and time and also Received: July 12, 2017. Revised: October 2, 2017. Accepted: November 18, 2017 © The Author 2018. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018 28 M. Uthman and M. Kamran Features of acute disseminated encephalomyletis seen on antibiotic cover of IV Ceftriaxone 2 g BD. She was also started MRI (Fig. 1). on oral Anti Tuberculous drugs, however, only continued with The diagnosis of MS was ruled out by, the location of that treatment for 2 days and was abandoned on further lesions, and their enhancement pattern was not typical of MS thought process and discussion by the medical team. and CSF analysis showed no oligoclonal bands. Autoimmune workup revealed U1-RNP antibodies significantly CSF report revealed marginally elevated CSF glucose and positive 14.18U/ml and SS-B/LA antibodies marginally positive CSF protein, polymorphs and lymphocytes were all within nor- 1.20U/ml along with negative Anti dsDNA and ANA. mal range. Final diagnosis of one-and a-half plus syndrome was made, Viral markers, i.e. CSF PCR for HSV1, HSV2 and VZV were i.e. nine syndrome. reported negative. After 1 week treatment course (IV. Acyclovir, IV. Dexametha- MRA and MRV were unremarkable to show any CT disease sone, Right eye horizontal nystagmus disappeared, movement association. of left eye (that with complete opthalmoplegia) improved in all All required labs including connective tissue profile ANA, other gazes, and left facial palsy also significantly improved. Anti-DsDNA, ENA profile sent and CSF examination including The patient could not walk without support but tendency to fall PCR for VZ, and HSV sent to Agha Khan lab. reduced significantly. She was empirically started on IV Acyclovir 750 mg TDS, Inj MRI scan after 1 week showed reduction in size as well as Dexamethasone, 4 mg QDS, IV Mannitol 100 ml × BD and mass effect of previously noted hyperactive intensities of brain and brain stem (Fig. 2). Patient was discharged from the hospital after 2 weeks course of antiviral on low dose steroidal therapy. She was no longer irritable, facial palsy, generalized weakness had improved and left eye lateral movement was marginally better. Counseling was done in detail. Patient visits the outdoor department every 4 weeks. She has showed complete recovery. No signs of facial palsy, left eye gaze palsy in any direction nor generalized weakness pre- sent. Currently she is on steroid sparing immunomodulators, i.e. Azathioprine 100 mg/day, Hydroxychloroquine 200 mg BD and Vitamin B6 along with physiotherapy. Regular follow up advised. DISCUSSION OAHS, Clinical classification based on neuro- ophthalmological manifestations (Can be classified if any one of the following fulfills the criteria with the patient) Figure 1: MRI brain (with gadolinium contrast) of patient on presentation showed multifocal hyper intensities in cortical and subcortical location of OAHS, Type 1 a. CHGP and INO (horizontal nystagmus) parietal, temporal, frontal lobes, basal ganglia, thalami, pons and left side b. CHGP and preserved abduction in one eye midbrain causing localized swelling, mild compression upon frontal horns of lateral ventricles. Ring like enhancements in some lesions of basal ganglia, Continued thalami and pons Figure 2: Comparison of MRI before and after 1 week treatment. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Case report of a patient with ‘one-and-a-half plus syndrome’ 29 Continued 16 Syndrome (Bilateral seventh nerve palsy + one-and-a-half syndrome + OAHS, Type II a. CHGP and normal adduction in one eye hemiparesis) [7]. b. Adduction nystagmus and preserved If simple arithmetic combinations adds confusion than clar- bilateral abductor ity, then adding the term ‘plus’ with typical OAHS, ‘Type I–II–III’ c. CHGP and bilateral abduction palsy may define the extent of illness. d. Any combination plus bilateral miosis OAHS, Type III a. CVGP and asymmetrical vertical eye movements CONFLICT OF INTEREST STATEMENT b. CVGP and vestibular nystagmus No conflict of interest. c. Vertical and horizontal gaze nystagmus at the same time FUNDING AN = Abducent nucleus. CHGB = Conjugate horizontal gaze palsy. No sources of funding. CVGP = Conjugate vertical gaze palsy. One-and-half plus syndromes ETHICAL APPROVAL Eight-and-half syndrome No approval required. OAHS with facial palsy due to posterior circulation syndrome, Eric Eggenberg [4] added cranial nerve 7 to OAHS (1½ + 7) and he named the combination as eight-and-half (8½) syndrome. CONSENT Other causes of this combination syndrome autoimmune A verbal and written informed consent was taken from the causes, hemorrhagic stroke, giant cell arteritis and pontine patient for writing of her case report. tuberculoma. More combinations are made by adding numbers of the cra- nial nerves involved, e.g.: REFERENCES 1. Karatas M. Internuclear and supranuclear disorders of eye movements: clinical features and causes. Eur J Neurol 2009; Five-and-a-half syndrome 16:1265–77. (1½ + 4-throclear nerve): OAHS and fourth nerve palsy. 2. Wall M, Wray SH. The one-and-a-half syndrome a unilateral disorder of the pons tegmentum: a study of 20 cases and review of the literature. Neurology 1983;33:971–80. Seven-and-a-half syndrome 3. Martin PJ, Chang HM, Wityk R, Caplan LR. Midbrain infarc- (1½ + 6-abducens): OAHS and sixth nerve palsy. tion: associations and aetiologies in the New England Medical Center Posterior Circulation Registry. J Neurol Nine syndrome Neurosurg Psychiatry 1998;64:392–5. 4. De Seze J, Lucas C, Leclerc X, Sahli A, Vermersch P, Leys D. Eight-and-a-half syndrome and an additional lesion in the mid- brain tegmentum/red nucleus causing contralesional hemia- One-and-a-half syndrome in pontine infarcts: MRO corre- lates. Neuroradiology 1999;41:666–9. taxia [6]. 5. Minagar A, Schatz NJ, Glaser JS. Case report: one-and-a-half syndrome and tuberculosis of the pons in a patient with 11½ Syndrome AIDS. AIDS Patient Care STDS 2000;14:461–4. (1½ + 7-facial nerve + 3-oculomotor nerve). 6. Rosini F, Pretegiani E, Guideri F, Cerase A, Rufa A. A eight and a half syndrome with hemiparesis and hemihypesthesia: the nine syndrome? J Stroke Cerebrovasc Dis 2003;22:637–8. 15½ Syndrome 7. Connors R, Ngan V, Howard J. A case of complete lateral gaze (Bilateral seventh nerve palsy and one-and-a-half syndrome: 7 + paralysis and facial diplegia: the 16 syndrome. J Neuroophthal- 7 + 1½). mol 2012;33:69–70. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018 http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Oxford Medical Case Reports Oxford University Press

Case report of a patient with ‘one-and-a-half plus syndrome: nine syndrome’

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Abstract

This case talks about ‘One-and-a-half plus syndrome’, a clinical syndrome affecting binocular vision and facial nerve. One-and-a- half plus syndrome is a less known clinical syndrome which constitutes of a conjugate horizontal gaze palsy in one direction and an internuclear ophthalmoplegia in the other direction. Despite the known association between ischemia, autoimmune disorders, multiple sclerosis, with mono neuritis multiplex resulting in extra ocular movement disorder, one-and-a-half plus syndrome is rarely considered in the differential diagnosis of eye ball movement disorders, as many clinicians are not able to diagnose such a case as ‘ the eyes don’tsee what theminddoesn’tknow’. Our report aims to raise awareness about connective tissue disorders presenting as neuro-ophthalmological syndrome, as early recognition can accelerate diagnosis and decrease the morbidity. had a ‘static left eye’ The family gave history of intermittent INTRODUCTION high grade fever, with profuse vomiting. The one-and-a half syndrome (1 and ½, OAHS) is a neurological On examination, patient was restless and irritable: she had disorder resulting in disorganization of conjugate eye movement: blood pressure of 110/60 mmHg, with no postural drop; pulse exotropia, conjugate horizontal gaze palsy (CHGP) in one direc- 90 b/min, respiratory rate 22 b/min and temperature 37.8°C. Oral tion and internuclear ophthalmoplegia (INO) in the other [1–5]. ulcers were present. During neurological examination she had Conjugate Horizontal gaze palsy (CHGP) and internuclear right side hemiataxia although there was not any focal motor def- opthalmoplegia/nystagmus is present if there is extensive icit. Cerebellar examination was unremarkable. Gag reflex present lesion involving the centers and tracts (medial longitudinal fas- on both sides. Brudzinski’s sign was positive and cranial nerve iculus) controlling binocular vision. examination revealed that the left eye could not move superiorly, The lesion may be infarction, atherosclerosis, hemorrhage inferiorly, medially nor laterally and there was right eye lateral in the basilar artery territory, demyelination, tuberculoma or gaze nystagmus. Left side facial palsy was also present. any other SOL. A clinical diagnosis of ‘One-and-a-half syndrome along with left side facial palsy’ was made and keeping in view of the pos- sible etiologies, she was planned to be worked up extensively, i.e CASE DESCRIPTION on lines of viral, bacterial, infectious and autoimmune etiologies. Nonspecific routine Laboratory tests revealed: hemoglobin A 16-year-old female was referred from a tertiary care hospital for steady gait, tendency to fall on right side,vertigo right sided 9.1 mg/dl, low HCT 31%, MCV 64 fl, MCHC 39 g/dl, MCH 19 pg, ESR 32 mm/h, platelets 331 × 10 /l, WBC 7.6. facial deviation, disorientation to persons and time and also Received: July 12, 2017. Revised: October 2, 2017. Accepted: November 18, 2017 © The Author 2018. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/ licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018 28 M. Uthman and M. Kamran Features of acute disseminated encephalomyletis seen on antibiotic cover of IV Ceftriaxone 2 g BD. She was also started MRI (Fig. 1). on oral Anti Tuberculous drugs, however, only continued with The diagnosis of MS was ruled out by, the location of that treatment for 2 days and was abandoned on further lesions, and their enhancement pattern was not typical of MS thought process and discussion by the medical team. and CSF analysis showed no oligoclonal bands. Autoimmune workup revealed U1-RNP antibodies significantly CSF report revealed marginally elevated CSF glucose and positive 14.18U/ml and SS-B/LA antibodies marginally positive CSF protein, polymorphs and lymphocytes were all within nor- 1.20U/ml along with negative Anti dsDNA and ANA. mal range. Final diagnosis of one-and a-half plus syndrome was made, Viral markers, i.e. CSF PCR for HSV1, HSV2 and VZV were i.e. nine syndrome. reported negative. After 1 week treatment course (IV. Acyclovir, IV. Dexametha- MRA and MRV were unremarkable to show any CT disease sone, Right eye horizontal nystagmus disappeared, movement association. of left eye (that with complete opthalmoplegia) improved in all All required labs including connective tissue profile ANA, other gazes, and left facial palsy also significantly improved. Anti-DsDNA, ENA profile sent and CSF examination including The patient could not walk without support but tendency to fall PCR for VZ, and HSV sent to Agha Khan lab. reduced significantly. She was empirically started on IV Acyclovir 750 mg TDS, Inj MRI scan after 1 week showed reduction in size as well as Dexamethasone, 4 mg QDS, IV Mannitol 100 ml × BD and mass effect of previously noted hyperactive intensities of brain and brain stem (Fig. 2). Patient was discharged from the hospital after 2 weeks course of antiviral on low dose steroidal therapy. She was no longer irritable, facial palsy, generalized weakness had improved and left eye lateral movement was marginally better. Counseling was done in detail. Patient visits the outdoor department every 4 weeks. She has showed complete recovery. No signs of facial palsy, left eye gaze palsy in any direction nor generalized weakness pre- sent. Currently she is on steroid sparing immunomodulators, i.e. Azathioprine 100 mg/day, Hydroxychloroquine 200 mg BD and Vitamin B6 along with physiotherapy. Regular follow up advised. DISCUSSION OAHS, Clinical classification based on neuro- ophthalmological manifestations (Can be classified if any one of the following fulfills the criteria with the patient) Figure 1: MRI brain (with gadolinium contrast) of patient on presentation showed multifocal hyper intensities in cortical and subcortical location of OAHS, Type 1 a. CHGP and INO (horizontal nystagmus) parietal, temporal, frontal lobes, basal ganglia, thalami, pons and left side b. CHGP and preserved abduction in one eye midbrain causing localized swelling, mild compression upon frontal horns of lateral ventricles. Ring like enhancements in some lesions of basal ganglia, Continued thalami and pons Figure 2: Comparison of MRI before and after 1 week treatment. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018 Case report of a patient with ‘one-and-a-half plus syndrome’ 29 Continued 16 Syndrome (Bilateral seventh nerve palsy + one-and-a-half syndrome + OAHS, Type II a. CHGP and normal adduction in one eye hemiparesis) [7]. b. Adduction nystagmus and preserved If simple arithmetic combinations adds confusion than clar- bilateral abductor ity, then adding the term ‘plus’ with typical OAHS, ‘Type I–II–III’ c. CHGP and bilateral abduction palsy may define the extent of illness. d. Any combination plus bilateral miosis OAHS, Type III a. CVGP and asymmetrical vertical eye movements CONFLICT OF INTEREST STATEMENT b. CVGP and vestibular nystagmus No conflict of interest. c. Vertical and horizontal gaze nystagmus at the same time FUNDING AN = Abducent nucleus. CHGB = Conjugate horizontal gaze palsy. No sources of funding. CVGP = Conjugate vertical gaze palsy. One-and-half plus syndromes ETHICAL APPROVAL Eight-and-half syndrome No approval required. OAHS with facial palsy due to posterior circulation syndrome, Eric Eggenberg [4] added cranial nerve 7 to OAHS (1½ + 7) and he named the combination as eight-and-half (8½) syndrome. CONSENT Other causes of this combination syndrome autoimmune A verbal and written informed consent was taken from the causes, hemorrhagic stroke, giant cell arteritis and pontine patient for writing of her case report. tuberculoma. More combinations are made by adding numbers of the cra- nial nerves involved, e.g.: REFERENCES 1. Karatas M. Internuclear and supranuclear disorders of eye movements: clinical features and causes. Eur J Neurol 2009; Five-and-a-half syndrome 16:1265–77. (1½ + 4-throclear nerve): OAHS and fourth nerve palsy. 2. Wall M, Wray SH. The one-and-a-half syndrome a unilateral disorder of the pons tegmentum: a study of 20 cases and review of the literature. Neurology 1983;33:971–80. Seven-and-a-half syndrome 3. Martin PJ, Chang HM, Wityk R, Caplan LR. Midbrain infarc- (1½ + 6-abducens): OAHS and sixth nerve palsy. tion: associations and aetiologies in the New England Medical Center Posterior Circulation Registry. J Neurol Nine syndrome Neurosurg Psychiatry 1998;64:392–5. 4. De Seze J, Lucas C, Leclerc X, Sahli A, Vermersch P, Leys D. Eight-and-a-half syndrome and an additional lesion in the mid- brain tegmentum/red nucleus causing contralesional hemia- One-and-a-half syndrome in pontine infarcts: MRO corre- lates. Neuroradiology 1999;41:666–9. taxia [6]. 5. Minagar A, Schatz NJ, Glaser JS. Case report: one-and-a-half syndrome and tuberculosis of the pons in a patient with 11½ Syndrome AIDS. AIDS Patient Care STDS 2000;14:461–4. (1½ + 7-facial nerve + 3-oculomotor nerve). 6. Rosini F, Pretegiani E, Guideri F, Cerase A, Rufa A. A eight and a half syndrome with hemiparesis and hemihypesthesia: the nine syndrome? J Stroke Cerebrovasc Dis 2003;22:637–8. 15½ Syndrome 7. Connors R, Ngan V, Howard J. A case of complete lateral gaze (Bilateral seventh nerve palsy and one-and-a-half syndrome: 7 + paralysis and facial diplegia: the 16 syndrome. J Neuroophthal- 7 + 1½). mol 2012;33:69–70. Downloaded from https://academic.oup.com/omcr/article-abstract/2018/1/omx085/4824926 by Ed 'DeepDyve' Gillespie user on 16 March 2018

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Published: Jan 1, 2018

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