Vertical gaze palsy

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Background: The aim is to evaluate the long-term efficacy of inferior oblique (IO) myectomy combined with Tenon's capsule closure to prevent muscle reattachment to the sclera. Methods: We retrospectively reviewed the medical records of 18 patients with primary and secondary IO overaction who underwent IO myectomy accompanied by Tenon's capsule closure. Patients were followed up for at least 1 year after the surgery. The main outcome measures included oblique muscle dysfunction, which was objectively graded through computerized analysis of nine-gaze photographs, and the amount of vertical deviation in the primary position using alternate prism cover testing...

We report a 63-year-old female patient with progressive supranuclear palsy (PSP). She presented predominant postural instability and "saccadic ping-pong gaze" (SPPG). She had unprovoked falls recurrently within a year from the onset of gait disturbance. She tended to fall backward with eye closure but had no freezing of gait on examination. She showed no signs of nuchal dystonia, limb tremor, rigidity, spasticity, or ataxia. The dopaminergic response was negative. On the initial examination, her vertical eye movements were normal, but frequent macro square wave jerks and SPPG were observed...

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by early postural instability and falls, oculomotor dysfunction (vertical supranuclear gaze palsy), parkinsonism with poor response to levodopa, pseudobulbar palsy, and cognitive impairment. This four-repeat tauopathy is morphologically featured by accumulation of tau protein in neurons and glia causing neuronal loss and gliosis in the extrapyramidal system associated with cortical atrophy and white matter lesions. Cognitive impairment being frequent in PSP and more severe than in multiple system atrophy and Parkinson disease, is dominated by executive dysfunction, with milder difficulties in memory, and visuo-spatial and naming dysfunctions...

BACKGROUND: POLR3A pathogenic variants are associated with hypomyelination, hypodontia, hypogonadism, and movement disorders. CASES: We describe the range of movement disorders seen in six patients (four female, two male) with POLR3A variants [three novel (c.2214del, c.3775G>A, c.3905G>T) and six previously reported (c.760C>T, c.1771-7C>G, c.1909+22G>A, c.2005C>T, c.2422C>T, c.3337-11T>C)]. Patient 1 presented with a neonatal progeroid syndrome and developed parkinsonism, dystonia, ataxia, and spasticity...

BACKGROUND: Corticobasal degeneration (CBD) may have a rapidly progressive (RP) clinical course, mimicking other neurological conditions. OBJECTIVES: To describe a neuropathologically proven case of RP-CBD in a patient initially diagnosed with immune-mediated brainstem encephalitis. METHODS: Retrospective data collection from electronic records and authorized video material. RESULTS: A 51-year-old man presented with bilateral ptosis, diplopia, and dysphagia...

PURPOSE: Occlusion of artery of Percheron (AOP), a rare variant of paramedian branches of posterior cerebral artery, results in a characteristic pattern of ischemic lesions in bilateral paramedian thalami with or without midbrain and anterior thalami involvement. AIM: To evaluate the prevalence, the clinical, and the imaging features of AOP infarction in a single comprehensive stroke center experience. METHODS: We retrospectively search in our stroke center database, patients with ischemic lesions in the AOP distribution...

BACKGROUND: Given the variable nature of clinical manifestations, neuronal intranuclear inclusion disease (NIID) is regarded as a heterogeneous disease which is challenging to diagnose early. To the present, progressive supranuclear palsy (PSP)-like symptoms have never been listed in the performance of NIID. CASE PRESENTATION: A 58-year-old man presented with progressive Parkinsonism and postural instability for 3 years. Initially, he was considered as probable PSP due to vertical supranuclear gaze palsy, postural instability, and hummingbird sign...

The chief goal of all eye movements is to maintain the image of an object steady on the retina especially the macula to preserve visual acuity. Gaze palsy refers to lack of the conjugate movements due to a failure of supranuclear control mechanisms. Supranuclear control is maintained by not one, but multiple eye movement systems and gaze mechanisms. Supranuclear gaze palsies can be associated with a myriad of aetiologies- from trauma or metabolic abnormalities to stroke, demyelinating disorders and space occupying lesions like tumours...

The artery of Percheron (AOP) is a variant of the posterior cerebral circulation where a single branch of either posterior cerebral artery supplies both paramedian territories of the thalami. A stroke of the AOP has become a neurodiagnostic conundrum due to its relative rarity and vague symptoms, and, hence, a missed opportunity for recanalization treatment. The classical presentation of AOP stroke is the triad of altered mental status, vertical gaze palsy, and memory impairment. Here, we describe a retrospective case review of a 59-year-old male presenting with confusion and slurred speech with subsequent symptoms such as blepharospasm and bradyphrenia...

BACKGROUND: Progressive supranuclear palsy (PSP) is a rapidly progressive primary tauopathy characterized by vertical gaze palsy, postural instability, and mild dementia. PSP shows high clinical and pathologic heterogeneity. Although a few risk factors exist, such as advanced age and environmental toxins, the precise etiology remains largely elusive. Compelling evidence now suggests that genetic background plays a pivotal role in the pathogenetic pathways of PSP. Notably, PSP is genetically and phenotypically a complex disorder...

No abstract text is available yet for this article.

INTRODUCTION: Vertical supranuclear gaze palsy is a key feature of Niemann-Pick type C (NP-C) and is commonly quantified using video-oculography (VOG). VOG requires sitting still for long times and performing specific tasks, thus it can be challenging or impossible for patients severely affected by movement disorders or cognitive impairment. To overcome this limitation, we measure saccades of NP-C patients using a fast eye tracking test based on continuous psychophysics and compare it to VOG...

INTRODUCTION: Niemann-Pick disease type C (NPC) is an autosomal recessive neurovisceral lipid storage disorder caused by mutations in the NPC 1 or 2 genes. Movement disorders can occur as the first symptom and as predominant symptom mainly in juvenile-onset. The frequency and heterogeneity of movement disorders in NPC are not well described. We studied the frequency and spectrum of movement disorders in patients with NPC of different age of onset. METHODS: Retrospective chart review of patients with NPC diagnosed based on the Suspicion Index tool and demonstration of foamy macrophages/sea-blue histiocytes in bone marrow aspirate...

Saccadic slowing as a component of supranuclear saccadic gaze palsy is an important diagnostic sign in multiple neurologic conditions, including degenerative, inflammatory, genetic, or ischemic lesions affecting brainstem structures responsible for saccadic generation. Little attention has been given to the accuracy with which clinicians correctly identify saccadic slowing. We compared clinician (n = 19) judgements of horizontal and vertical saccade speed on video recordings of saccades (from 9 patients with slow saccades, 3 healthy controls) to objective saccade peak velocity measurements from infrared oculographic recordings...

BACKGROUND: Paraneoplastic limbic encephalitis is a rare neurologic syndrome that affects patients with cancer and commonly presents with symptoms of personality changes, visual disturbances, seizures, vertigo, and memory loss. It has an immune-mediated pathophysiology and is associated with antibodies directed against both the tumor and limbic structures in the nervous system. Here, we report a case of paraneoplastic limbic encephalitis with an unusual presentation with initial symptoms of panhypopituitarism...

A 58-year-old previously healthy woman presents with 3 years of rapidly progressive ataxia, parkinsonism, dysautonomia, peripheral neuropathy, leg weakness, spasticity, hyperreflexia, and mild vertical-gaze palsy. She has a matrilineal family history of neurodegenerative diseases. She was initially postulated to have spinocerebellar ataxia or atypical parkinsonism with cerebellar features. However, on closer inspection, her abnormal extraocular eye movements suggested rare mimicking disorders such as prion disease as part of the differential diagnosis, requiring further evaluation...

SQSTM1 gene encodes a protein called p62 that acts as an autophagy receptor in the degradation of protein molecules. A homozygous deletion variant that changes the frame shift in the SQSTM1 gene named c.790 Del A .T was detected in case childhood onset and progressive neurodegeneration with ataxia, and gaze palsy.

BACKGROUND: to report the results of augmented inferior rectus muscle transposition (IRT) in management of chronic sixth nerve palsy. METHODS: a retrospective review of medical records of patients with chronic complete sixth nerve palsy who were treated by augmented full thickness IRT to the lateral border of the paralyzed lateral rectus muscle. Patients were selected for IRT if there was more limitation of abduction in inferior gaze associated with V- pattern esotropia...

Vertical pendular nystagmus (PN) rarely occurs with acute pontine lesions. To hypothesize a pathophysiology for acute vertical PN, we analyzed the clinical characteristics and quantitative eye-movement recordings of one new case with acute vertical PN and an additional 11 patients from the literature. Most patients had extensive pontine lesions causing either the locked-in syndrome or unresponsiveness, but two conscious patients had focal lesions restricted to the paramedian caudal pontine tegmentum. All patients presented a complete or partial horizontal gaze palsy, and about half showed ocular bobbing before or during the appearance of vertical PN...