Freezing of saccades in dopa-responsive parkinsonian syndrome

Abstract

Purpose Ocular motor abnormalities such as abnormal saccades are common in idiopathic Parkinson's disease (PD) and atypical parkinsonian syndrome, such as progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD). In this study, we describe a case of patient with PD and show a video illustrating severe delay of reflexive saccades. Observations A 68-year-old Caucasian woman with diagnosis of PD presented for evaluation of diplopia. Neuro-ophthalmic examination revealed good visual acuity in both eyes and normal optic nerves but prominent ocular motor abnormalities, including hypometric saccades, impaired smooth pursuit, and convergence insufficiency causing diplopia at near. Despite treatment with carbidopa-levodopa three times per day, she exhibited episodic, severe delay of reflexive saccades. During these episodes, the patient appeared frozen and unable to initiate reflexive saccades for 20 s or longer. This freezing of reflexive saccades was variable and occurred suddenly during exam but could be interrupted by smooth pursuit. There was no gait freezing, eyelid apraxia, or prominent exacerbation of other motor symptoms. Freezing of saccades dramatically resolved after increasing dosage of carbidopa-levodopa. Conclusions and Importance We describe a patient with dopa-responsive parkinsonian syndrome with intermittent difficulty initiating reflexive saccades mimicking ocular motor apraxia. Resolution of saccadic freezing with higher carbidopa-levodopa is consistent with ocular motor impairment as a result of degeneration and dysfunction of the dopaminergic pathways in supranuclear ocular motor control.

Diplopia in Movement Disorders: A Systematic Review of the Literature

“…The rest of the articles were manually screened by the authors in order to meet the eligibility criteria. In the end, a total of seventeen articles remained: nine were observational cross-sectional studies [ 3 , 4 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ], two were prospective cohort studies [ 13 , 14 ], two were retrospective studies [ 15 , 16 ] and four were case reports [ 17 , 18 , 19 , 20 ]. Table 1 shows detailed information about the included studies on diplopia in Parkinson’s disease.…”

“…One study compared patients with PD, PD dementia, and healthy controls [ 11 ], two studies compared PD patients with diplopia, PD patients without diplopia, and healthy controls (HC) [ 12 , 13 ], four studies examined patients with PD compared to healthy controls [ 4 , 10 , 13 , 14 ], six studies examined PD patients with and without diplopia [ 3 , 6 , 7 , 8 , 15 , 16 ], and four were case reports of PD patients with diplopia [ 17 , 18 , 19 , 20 ].…”

“…Mechanisms of diplopia are heterogenous and include convergence insufficiency [ 6 , 15 , 17 ], strabismus, ocular pathology as well as other mechanisms related to motor fluctuations [ 18 ], the presence of visual hallucinations [ 3 , 4 , 6 , 7 , 8 , 12 , 13 ], and the treatment of Parkinson’s disease [ 8 , 19 , 20 ].…”

“…Diplopia has also been linked to motor “off” periods and motor fluctuations in some patients [ 17 , 18 ] and to the treatment of the disease [ 8 , 19 , 20 ]. Likitgorn T. et al reported the case of a patient with an 8-year history of PD who developed diplopia.…”

Language Disorder in Progressive Supranuclear Palsy and Corticobasal Syndrome: Neural Correlates and Detection by the MLSE Screening Tool

“…However, there are overlapping neuropathological features including neuronal, oligodendroglial, and astrocytic inclusions that are immunoreactive for tau with 4 microtubule binding repeats (4R; Grossman, 2010;Dickson et al, 2011;Spinelli et al, 2017). PSP and CBS clinical signs often follow the presentation of nfvPPA (Kertesz and McMonagle, 2010;Santos-Santos et al, 2016;Cerami et al, 2017;Gazzina et al, 2019), or primary progressive apraxia of speech (Josephs et al, 2014). The clinical and pathological overlap of PSP, CBS, and nfvPPA underlies the concept of a continuous spectrum of 4R-Tauopathy disorders (Kertesz et al, 2005;Dickson et al, 2011;Murley et al, 2020) that extends to the functional anatomy of their cognitive deficits.…”