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How Do I Examine for a Supranuclear Gaze Palsy?

ABSTRACT: Classically, a supranuclear gaze palsy (SNGP) is a conjugate gaze limitation that can be overcome (i.e., corrected) by the vestibulo-ocular reflex (VOR). It involves vertical eye movement most commonly. In practice, SNGP can be encountered as a limitation in excursion of self-generated conjugate saccades (fast eye movements) that can be improved or overcome with verbal command, visual targets, or VOR. Thus, mild SNGPs may be overcome with verbal commands (e.g., "look down") or visual targets (e.g., "look down at my hand"), whereas moderate SNGPs may be overcome by smooth pursuit mechanisms and following a visual target (e.g., "look at my pen as it moves") and severe SNGPs can only be overcome with the VOR (i.e., fixating straight ahead while the head is passively rotated). So, it is best to assess for an SNGP in a hierarchical fashion; initially assessing saccades to command, then saccades to visual targets, then smooth pursuit of a target, and finally with the VOR.

SUBMITTER: Anderson T

PROVIDER: S-EPMC6353330 | biostudies-literature | 2015 Mar

REPOSITORIES: biostudies-literature

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How Do I Examine for a Supranuclear Gaze Palsy?

Anderson Tim T

Movement disorders clinical practice 20141206 1

Classically, a supranuclear gaze palsy (SNGP) is a conjugate gaze limitation that can be overcome (i.e., corrected) by the vestibulo-ocular reflex (VOR). It involves vertical eye movement most commonly. In practice, SNGP can be encountered as a limitation in excursion of self-generated conjugate saccades (fast eye movements) that can be improved or overcome with verbal command, visual targets, or VOR. Thus, mild SNGPs may be overcome with verbal commands (e.g., "look down") or visual targets (e. ...[more]

PMID: 30713889

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Pathologic correlates of supranuclear gaze palsy with parkinsonism.

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Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos.

Project description:Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.

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Association of Progressive Supranuclear Palsy Rating Scale with Progressive Supranuclear Palsy Quality of Life Scale.

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| S-EPMC8119324 | biostudies-literature

Genetics of Progressive Supranuclear Palsy.

Project description:Progressive supranuclear palsy (PSP) is a neurodegenerative syndrome that is clinically characterized by progressive postural instability, supranuclear gaze palsy, parkinsonism and cognitive decline. Pathologically, diagnosis of PSP is based on characteristic features, such as neurofibrillary tangles, neutrophil threads, tau-positive astrocytes and their processes in basal ganglia and brainstem, and the accumulation of 4 repeat tau protein. PSP is generally recognized as a sporadic disorder; however, understanding of genetic background of PSP has been expanding rapidly. Here we review relevant publications to outline the genetics of PSP. Although only small number of familial PSP cases have been reported, the recognition of familial PSP has been increasing. In some familial cases of clinically probable PSP, PSP pathologies were confirmed based on NINDS neuropathological diagnostic criteria. Several mutations in MAPT, the gene that causes a form of familial frontotemporal lobar degeneration with tauopathy, have been identified in both sporadic and familial PSP cases. The H1 haplotype of MAPT is a risk haplotype for PSP, and within H1, a sub-haplotype (H1c) is associated with PSP. A recent genome-wide association study on autopsyproven PSP revealed additional PSP risk alleles in STX6 and EIF2AK3. Several heredodegenerative parkinsonian disorders are referred to as PSP-look-alikes because their clinical phenotype, but not their pathology, mimics PSP. Due to the fast development of genomics and bioinformatics, more genetic factors related to PSP are expected to be discovered. Undoubtedly, these studies will provide a better understanding of the pathogenesis of PSP and clues for developing therapeutic strategies.

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Project description: Not available

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Immunotherapy in progressive supranuclear palsy.

Project description:Purpose of reviewProgressive supranuclear palsy (PSP) is a progressive adult-onset neurodegenerative disease. Abnormally, phosphorylated forms of the microtubule-associated protein tau containing four repeat domains (4R-tau) aggregate in neurons. Additionally, increasing evidence suggests that secretion and uptake of fragments of abnormal 4R-tau may play a role in disease progression. This extracellular tau is a natural target for immunotherapy.Recent findingsThree monoclonal antibodies targeting extracellular tau are in clinical stages of development. ABBV-8E12 and BIIB092 were safe in Phase 1, but both Phase two studies recently failed futility analyses. UCB0107 recently reported (in abstract form) Phase 1 safety results, and a Phase 2 study is under consideration. Stem cell therapy and the infusion of plasma are also being explored clinically.SummaryThe likely role of extracellular tau in the progression of PSP makes tau a natural target for targeted immunotherapy. Clinical trials are still in early stages, and although tau immunotherapy has largely been shown to be safe, efficacy has yet to be demonstrated.

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Tremor in progressive supranuclear palsy.

Project description:IntroductionTremor is thought to be a rare feature of progressive supranuclear palsy (PSP).MethodsWe retrospectively reviewed the database of the CurePSP brain bank at Mayo Clinic Florida to retrieve all available clinical information for PSP patients. All patients underwent a standard neuropathological assessment and an immunohistochemical evaluation for tau and α-synuclein. DNA was genotyped for the MAPT H1/H2 haplotype.ResultsOf the 375 PSP patients identified, 344 had a documented presence or absence of tremor, which included 146 (42%) with tremor, including 29 (20%) with postural/action tremors, 16 (11%) with resting tremor, 7 (5%) with intention tremor, 20 (14%) with a combination of different types of tremor, and 74 (51%) patients who had tremor at some point during their illness, but details were unavailable. The tremor severity of 96% of the patients (54/55) who had this data was minimal to mild. The probability of observing a tremor during a neurological examination during the patient's illness was estimated to be ∼22%. PSP patients with postural/action tremors and PSP patients with resting tremor responded to carbidopa-levodopa therapy more frequently than PSP patients without tremor, although the therapy response was always transient. There were no significant differences in pathological findings between the tremor groups.ConclusionsTremor is an inconspicuous feature of PSP; however, 42% (146/344) of the PSP patients in our study presented some form of tremor. Because there is no curative therapy for PSP, carbidopa/levodopa therapy should be tried for patients with postural, action, and resting tremor.

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