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We report, to the best of our knowledge, the first case of neuropsychiatric systemic lupus erythematosus with clinical presentation of bilateral upward gaze palsy and intraoral numbness. Magnetic resonance imaging of the brain was able to identify the pathogenic lesion at the left side of midbrain, involving the vertical gaze center and sensory pathways for innervating the buccal and hard palate mucosa. A course of aggressive immunosuppressive treatment resulted in prompt resolution of gaze palsy and the midbrain lesion.

Bilateral upward and ipsilateral downward gaze palsy due to a unilateral thalamomesencephalic stroke is called vertical one‐and‐a‐half syndrome (VOHS). Here, we report a valiant VOHS case who presented contralateral upward and ipsilateral downward gaze palsy due to a unilateral thalamomesencephalic stroke. The neuronal fiber connections associated with vertical gaze are not completely understood, so the present case provides an important proof to obtain a better understanding of vertical gaze mechanisms.

Introduction: Parinaud syndrome, caused by midbrain infarction, usually manifests as an ocular conjugate upgaze palsy. However, this sign should not point out straightforwardly to Parinaud syndrome, as other lesions in the central nervous system could cause it. Case description: The case of a 47-year-old woman showing acute onset of diplopia with bilateral upward gaze palsy is described. Parinaud syndrome was suspected on clinical grounds. However, brain magnetic resonance imaging displayed an acute ischemic lesion in the right anteromedial thalamus. Conclusions: Bilateral upward gaze palsy may be caused by unilateral thalamic infarction. The mechanism by which a unilateral thalamic lesion causes bilateral gaze palsy is discussed.

Eye movement disorders are rarely reported in vitamin B12 deficiency. Reported eye movement disorders in vitamin B12 deficiency were downbeat nystagmus and INO in 3 cases each, upward gaze palsy in 2 cases, bilateral abducens palsy, total ophthalmoplegia, bilateral horizontal gaze evoked nystagmus in 1 case each. We describe a case with recently developed horizontal penduler constant nystagmus due to vitamin B12 deficiency. He received replacement therapy but his eye movement disorder did not respond to treatment. The early diagnosis of vitamin B12 deficiency is vital because when axonal damage is started recovery might not occur. To the best of our knowledge, this is the first reported case with acquired horizontal pendulernystagmus caused by vitamin B12 deficiency.

The authors describe three siblings born to consanguineous parents with early onset ataxia, dysarthria, myoclonic, generalized tonic clonic seizures, upward gaze palsy, extensor plantar reflexes, sensory neuropathy, and normal cognition. Direct screening excluded mutations in FRDA, TDP1, and SACS genes and at 8344, 3243, and 8993 positions of mitochondrial DNA. Linkage analysis excluded AOA-1, EPM1, EPM2A, EPM2B, CAMOS, and recessive ataxias linked to chromosome 9q34-9qter. This clinical constellation may represent a distinct form of early onset cerebellar ataxia.

Spontaneous abnormal eye movements are frequently caused by disease in the aqueduct of the midbrain and indicate the location of the lesion the at an early stage.We report here a case of Sylvian aqueduct syndrome in which neuro-otological findings became clues to the diagnosis. Our patient was a 54-year-old male with the chief complaint of unstable dizziness. He showed intermittent convergence nystagmus and upward gaze palsy. Hydrocephalus following non-tumoral aqueduct stenosis resulted in a lower inclination of the brainstem and caused such ocular symptoms. Structural changes could not be demonstrated in images at an early stage. Oculomotor abnormalties changed after a V-P shunt was placed and eventually disappeared.

Three males and one female with alternating skew deviation are reported. All of them showed adducting hypotropia in the lateral gaze for less than one month, which resolved. Duction was full in each direction. Three had brainstem tumor and one had a history of viral encephalitis. All of them had accompanying pretectal signs, such as upward gaze palsy, defective pupillary reaction, and nystagmus. Patients with alternating skew with pretectal signs may need urgent surgical intervention.

症例は57歳の女性.昭和63年2月, 意識障害をきたし近医に入院.頭部CT所見は水頭症を伴う脳室内出血であった.平成2年1月29日当科に精査入院し, MRIで右視床と側脳室の境界部に血腫の主座が確認された.高血圧の既往および明らかな動脈瘤, 動静脈奇形などの血管異常がないことから原発性脳室内出血と診断した.神経学的には, 発症時より上方注視麻痺, 下方注視時の垂直性眼振が持続して観察された.原発性脳室内出血では眼球運動障害を合併することが多いが, その頻度および発症機序は十分には明らかにされていない.本症における眼球運動障害の発現にはrostral interstitial nucleus of the medial longitudinal fasciculus (riMLF) の関与が示唆された.

Object. This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction.Methods. All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocisternostomy allowed resolution of the symptoms and withdrawal of the shunt.Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments, with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy.Conclusions. It is probable that in obstructive hydrocephalus, at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain. Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.

We found dyskinetic strabismus in 66 patients with cerebral palsy. The most striking feature of dyskinetic strabismus is the fluctuation from esotropia to exotropia under the same accommodative conditions with a slow tonic deviation similar to a vergence movement. With increasing age, exodeviation becomes more prevalent. Dyskinetic strabismus is seen exclusively in cerebral palsy patients. Many of these patients have an athetoid component to their disorder. The association of dyskinetic strabismus with athetosis and upward gaze palsy suggests that the basal ganglia may be the site of the malfunction. The strabismus responds poorly to surgery and the associated athetosis is important in the diagnosis and treatment of cerebral palsy.

São estudados dois pacientes com hidrocéfalo hipertensivo que apresentaram, no curso de sua doença, sindrome de Parinaud. Em ambos os casos o exame ventriculográfico, com contraste positivo, demonstrou a existência de dilatação cística do recesso suprapineal que foi imputada como causa da sindrome deficitária oculomotora. A literatura concernente é revisada, tendo sido salientada a importância da investigação radiológica cuidadosa da porção posterior do III ventriculo em pacientes com hipertensão endocraniana e síndrome de Parinaud. É comentado o diagnóstico radiológico diferencial. A importância do uso de válvulas corretamente manufaturadas e que coincidam com as especificações do seu fabricante é discutida. The cases of two patients with hypertensive hydrocephalus and Parinaud syndrome are reported. In both cases the ventriculographic studies showed a cystic expansion of the suprapineal recess. The upward gaze palsy was related with the expansion of the suprapineal recess. The radiological study of the posterior portion of the third ventricle in these cases is recomended. The use of inadequate devices in these situations may cause the worsing of the patient.