The “round the houses” sign and “zig-zag” sign in progressive supranuclear palsy and other conditions

Fearon, Conor; Field, Robert H.; Donlon, Eoghan; Murphy, Olwen C.; Cronin, Simon; Buckley, Cliona; Williams, Laura; Llamas, Yudy; O’Dowd, Seán; O’Sullivan, Sean S.; Gold, Daniel R.; Walsh, Richard A.; Healy, Daniel G.; Quinn, Niall; Lynch, Timothy

doi:10.1016/j.parkreldis.2020.10.030

Cited by 11 publications

(5 citation statements)

References 8 publications

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“…The association of young-adult-onset cerebellar ataxia, vertical supranuclear gaze palsy, bulbar dysfunction, and cognitive impairment suggest the diagnosis of NPC. 2 In our patient, molecular testing disclosed the homozygous variant NM_000271.4 (NPC1): c.2292G>A;(Silent), classified as pathogenic, and confirmed the diagnosis.…”

Section: What Is the Most Likely Diagnosis?supporting

confidence: 66%

“…The “round the houses” sign is the manifestation of an early stage of vertical supranuclear gaze palsy, caused by an abnormality in the rostral interstitial nucleus of medial longitudinal fasciculus (riMLF), in which an adaptive strategy occurs with a large horizontal component to prolong the time available to generate the vertical component. The sign can be seen in this patient especially in the downward gaze, whereas in the upward gaze it is possible to see random horizontal intrusions, the “zig‐zag” sign 2 …”

Section: Answermentioning

confidence: 69%

“…The sign can be seen in this patient especially in the downward gaze, whereas in the upward gaze it is possible to see random horizontal intrusions, the "zig-zag" sign. 2…”

Section: Questionsmentioning

confidence: 99%

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A Woman with Progressive Ataxia and Abnormal Eye Movements

Silva

Ferreira

Pedroso

et al. 2022

Movement Disord Clin Pract

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A 28-year-old woman presented with progressive gait ataxia and speech impairment that started 7 years ago. Parents were nonconsanguineous and family history was unremarkable. During the last 2 years, she developed sialorrhea and dysphagia. Recently, she was diagnosed with depression, and her cognition was mildly impaired. She did not have organomegaly, nor sleep disorders, and the magnetic resonance imaging (MRI) showed mildly enlarged ventricles. Neurological examination disclosed dysarthria, axial and appendicular cerebellar ataxia, neck and truncal dystonia with left tilt, reduced arm swing on the left, and abnormal ocular movements (Video 1).

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Section: What Is the Most Likely Diagnosis?supporting

confidence: 66%

Section: Answermentioning

confidence: 69%

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A Woman with Progressive Ataxia and Abnormal Eye Movements

Silva

Ferreira

Pedroso

et al. 2022

Movement Disord Clin Pract

View full text Add to dashboard Cite

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“…71 This points towards the involvement of brainstem and is supported by the presence of other indicators of brainstem dysfunction such as increased square wave jerks and gaze instability as seen in PSP. 72 High frequency and high amplitude square wave jerks are most prominent in PSP compared to other Parkinsonian syndromes and are mediated by the supratentorial cortical structures in the temporal lobe. 73 In a comparative study between amyotrophic sclerosis, PSP and controls, it was found that the occurrence of square wave jerks is a function of the amplitude of the saccadic intrusions rather than the group.…”

Section: Progressive Supranuclear Palsymentioning

confidence: 99%

Detecting Abnormal Eye Movements in Patients with Neurodegenerative Diseases – Current Insights

Sekar,

Panouillères,

Kaski

2024

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This review delineates the ocular motor disturbances across a spectrum of neurodegenerative disorders, including Alzheimer's Disease (AD) and related disorders (ADRD), Parkinson's Disease (PD), atypical parkinsonism, and others, leveraging advancements in eye-tracking technology for enhanced diagnostic precision. We delve into the different classes of eye movements, their clinical assessment, and specific abnormalities manifesting in these diseases, highlighting the nuanced differences and shared patterns. For instance, AD and ADRD are characterized by increased saccadic latencies and instability in fixation, while PD features saccadic hypometria and mild smooth pursuit impairments. Atypical parkinsonism, notably Progressive Supranuclear Palsy (PSP) and Corticobasal Syndrome (CBS), presents with distinct ocular motor signatures such as vertical supranuclear gaze palsy and saccadic apraxia, respectively. Our review underscores the diagnostic value of eye movement analysis in differentiating between these disorders and also posits the existence of underlying common pathological mechanisms. We discuss how eye movements have potential as biomarkers for neurodegenerative diseases but also some of the existing limitations.

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“…They occur in strokes as well as in metabolic diseases such as Niemann-Pick type C [7,8] or neurodegenerative diseases such as atypical parkinsonian types, e.g. progressive supranuclear palsy (PSP) [9].…”

Section: Rostral Nucleus Of the Medial Longitudinal Fasciculus (Rimlf)mentioning

confidence: 99%

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases

Strupp

Straumann

Helmchen

2021

Klin Monbl Augenheilkd

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The key to the diagnosis of ocular motor disorders is a systematic clinical examination of the different types of eye movements, including eye position, spontaneous nystagmus, range of eye movements, smooth pursuit, saccades, gaze-holding function, vergence, optokinetic nystagmus, as well as testing of the function of the vestibulo-ocular reflex (VOR) and visual fixation suppression of the VOR. This is like a window which allows you to look into the brain stem and cerebellum even if imaging is normal. Relevant anatomical structures are the midbrain, pons, medulla, cerebellum and rarely the cortex. There is a simple clinical rule: vertical and torsional eye movements are generated in the midbrain, horizontal eye movements in the pons. For example, isolated dysfunction of vertical eye movements is due to a midbrain lesion affecting the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), with impaired vertical saccades only or vertical gaze-evoked nystagmus due to dysfunction of the Interstitial nucleus of Cajal (INC). Lesions of the lateral medulla oblongata (Wallenberg syndrome) lead to typical findings: ocular tilt reaction, central fixation nystagmus and dysmetric saccades. The cerebellum is relevant for almost all types of eye movements; typical pathological findings are saccadic smooth pursuit, gaze-evoked nystagmus or dysmetric saccades. The time course of the development of symptoms and signs is important for the diagnosis of underlying diseases: acute: most likely stroke; subacute: inflammatory diseases, metabolic diseases like thiamine deficiencies; chronic progressive: inherited diseases like Niemann-Pick type C with typically initially vertical and then horizontal saccade palsy or degenerative diseases like progressive supranuclear palsy. Treatment depends on the underlying disease. In this article, we deal with central ocular motor disorders. In a second article, we focus on clinically relevant types of nystagmus such as downbeat, upbeat, fixation pendular, gaze-evoked, infantile or periodic alternating nystagmus. Therefore, these types of nystagmus will not be described here in detail.

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The “round the houses” sign and “zig-zag” sign in progressive supranuclear palsy and other conditions

Cited by 11 publications

References 8 publications

A Woman with Progressive Ataxia and Abnormal Eye Movements

A Woman with Progressive Ataxia and Abnormal Eye Movements

Detecting Abnormal Eye Movements in Patients with Neurodegenerative Diseases – Current Insights

Central Ocular Motor Disorders: Clinical and Topographic Anatomical Diagnosis, Syndromes and Underlying Diseases

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