"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases

Mahale, Rohan; Mehta, Anish; John, Aju Abraham; Javali, Mahendra; Abbas, Mirza Masoom; Srinivasa, Rangasetty

doi:10.4103/0972-2327.157180

Cited by 14 publications

(12 citation statements)

References 7 publications

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“…described a variation to nine syndrome in two patients who had eight and a half syndrome without hemiparesis/hemianesthesia but instead had contralateral hemiataxia due to the involvement of inferior cerebellar peduncle in one patient and red nucleus in another. [ 8 ] Our patient had hemiparesis and hemianesthesia similar to the patient first described by Rosini et al . The MRI brain showed two areas of diffuse restriction, one in the right paramedial pons and the second more caudal in the dorsal pons.…”

Section: Discussionsupporting

confidence: 77%

“…Nine syndrome is a rare entity which was reported by Rosini et al .,[ 7 ] and there have been only three cases reported in literature till date. [ 7 8 9 ] This syndrome comprises eight and a half syndrome associated with hemiparesis and hemianesthesia due to additional involvement of the corticospinal tract and medial lemniscus. Mahale et al .…”

Section: Discussionmentioning

confidence: 99%

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Nine syndrome: Case report and review of clinical signs in internuclear ophthalmoplegia

Singhdev

Asranna

Sureshbabu

et al. 2018

Ann Indian Acad Neurol

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Section: Discussionsupporting

confidence: 77%

Section: Discussionmentioning

confidence: 99%

Nine syndrome: Case report and review of clinical signs in internuclear ophthalmoplegia

Singhdev

Asranna

Sureshbabu

et al. 2018

Ann Indian Acad Neurol

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“…Mahale (19) reported two cases of nine syndrome in 2015: Case 2: a 62-year-old female, acute onset and symptoms persisted, manifested as the left side of one-and-a-half syndrome + ipsilateral peripheral facial paralysis + ipsilateral limb ataxia (the left finger-nose test and heel-knee-tibia test were less stable and the body is dumped to the left when walking in a straight line). Cerebrospinal fluid protein was slightly elevated, mononuclear cells were normal, and anti-NMO antibody and CSF-IgG were negative.…”

Section: Nine Syndromementioning

confidence: 99%

One-and-a-half syndrome with its spectrum disorders

Xue¹,

Zhang²,

Zhang³

et al. 2017

Quant. Imaging Med. Surg.

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One-and-a-half syndrome is a syndrome characterized by horizontal movement disorders of the eyeballs, which was first reported and named by Fisher in 1967. It presents a combination of ipsilateral conjugate horizontal gaze palsy (one) and ipsilateral internuclear ophthalmoplegia (INO) (a half). On the basis of the one-and-a-half syndrome, there are a series of related rare syndromes called the one-and-a-half syndrome spectrum disorders. This article reviews rare cases of one-and-a-half syndrome spectrum disorder, describes the clinical and pathological features of different syndromes, and summarizes their nomenclature. Quant Imaging Med Surg 2017;7(6):691-697 qims.amegroups.com Xue et al. One-and-a-half syndrome with its spectrum disorders horizontal conjugate movement of eyes. If the isolated side of PPRF is damaged, the eyes could not gaze at the lesion side, presenting the horizontal gaze palsy to the lesion side (6).The abducens nucleus is located in colliculus facialis, which is in the lower part of the pons and the bottom of the fourth ventricle (7). It contains two functionally distinct cell groups: the abducens motoneurons that innervate the lateral rectus muscle; the internuclear neurons which axons cross the midline and ascend via the contralateral MLF to oculomotor subnucleus and control the medial rectus (8). Therefore, the unilateral lesion of the abducens nucleus will also produce ipsilateral horizontal gaze paralysis.MLF are the longitudinal nerve fibers that locate on the back of the pons and are close to the front of the fourth ventricle, a part of which relating to horizontal gaze is from the side of the abducens nucleus to the contralateral oculomotor subnucleus (9). The lesion of unilateral pontine tegmentum can damage the ipsilateral MLF, causing the ipsilateral INO, and its clinical manifestations show the ipsilateral intraocular muscle paralysis and contralateral horizontal nystagmus when staring at the opposite side. If the damage does not involve the midbrain, the convergence reflex is integrated (10). One-and-a-half syndromeThe one-and-a-half syndrome is caused by a lesion of unilateral tegmentum of pons, causing damage to the PPRF (or abducens nucleus) and MLF ( Figure 2). The most common cause of one-and-a-half syndrome was cerebrovascular disease, and usually was brain stem lacunar infarction, followed by the demyelinating etiology (multiple sclerosis), and then the infectious cause including neurocysticercosis and brainstem encephalitis. Other uncommon causes were head trauma, brain stem tumor (primary or metastasis), astrocytomas, etc. (6,11). Therefore, the vast majority of one-and-a-half syndrome companied by other positioning signs is due to the different lesions of brainstem (3). Only a very small number of patients showed isolated one-and-a half syndrome, while the lesions are smaller and localized, such as brainstem cysticercosis, brainstem tuberculosis, brainstem cavernous hemangioma and the local hemorrhagic infarction of brainstem (12-15).We recently diagnosed a ...

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“…Until now, 18 cases of EHS have been reported and the etiology in most of them was ischemic infarction in dorsal tegmentum of pons. Other etiologies included infection in one (tuberculoma), 3 ICH in one, 2 giant cell arteritis in one , 4 and demyelination compatible with multiple sclerosis in three patients. 5 , 7 In our cases, case 1, 3, and 4 were EHS with ICH, demyelination, and NMO spectrum disorder etiologies.…”

Section: Discussionmentioning

confidence: 99%

“…Extension of lesion to adjacent structures such as corticospinal tract, medial lemniscus, or cerebellar peduncle may suggest nine syndrome which is recently proposed (8.5 + 0.5 = 9). 1 , 2 The combination of these clinical findings allows precise localization of the lesion, and therefore could have significant diagnostic value.…”

Section: Introductionmentioning

confidence: 99%

"Eight and a half" and "nine syndrome" rare presentation of pontine lesions; case reports and review of literature

Yadegari

Aghsaei-Fard

Akbari

et al. 2019

CJN

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Background: Eight-and-a-half syndrome (EHS) is one-and-a-half syndrome [(conjugated horizontal gaze palsy and internuclear ophthalmoplegia (INO)] plus ipsilateral fascicular seventh cranial nerve palsy. Involvement of lower pontine tegmentum including the abducens nucleus, the ipsilateral medial longitudinal fasciculus (MLF), and the adjacent facial colliculus contribute to the clinical findings of EHS. Recently, nine syndrome with addition of hemiparesis or hemianesthesia to EHS (due to involvement of adjacent corticospinal tract or medial lemniscus) is suggested. Methods: Consecutive patients with presentation of EHS or nine syndrome were reviewed from referral neuro-ophthalmology and strabismus clinics. Results: Three cases of EHS were identified with different etiologies of intracerebral hemorrhage (ICH), demyelination, and neuromyelitis optica spectrum disorder. Moreover, one case of "nine syndrome" due to ICH was described. Brain magnetic resonance imaging (MRI) in all of them revealed lesion in lower tegmentum of pons. Conclusion: Apart from different etiologies, recognition of EHS or nine syndrome allows precise localization of the lesion to lower pontine tegmentum ipsilaterally.

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"Nine" syndrome: A new neuro-ophthalmologic syndrome: Report of two cases

Cited by 14 publications

References 7 publications

Nine syndrome: Case report and review of clinical signs in internuclear ophthalmoplegia

Nine syndrome: Case report and review of clinical signs in internuclear ophthalmoplegia

One-and-a-half syndrome with its spectrum disorders

"Eight and a half" and "nine syndrome" rare presentation of pontine lesions; case reports and review of literature

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