Pathologic correlates of supranuclear gaze palsy with parkinsonism

Abstract

Introduction Supranuclear gaze palsy (SGP) is a classic clinical feature of progressive supranuclear palsy (PSP) but is not specific for this diagnosis and has been reported to occur in several other neurodegenerative parkinsonian conditions. Our objective was to evaluate the association between SGP and autopsy-proven diagnoses in a large population of patients with parkinsonism referred to a tertiary movement disorders clinic. Methods We reviewed clinical and autopsy data maintained in an electronic medical record from all patients seen in the Movement Disorders Clinic at Washington University, St. Louis between 1996 and 2015. All patients with parkinsonism from this population who had subsequent autopsy confirmation of diagnosis underwent further analysis. Results 221 unique parkinsonian patients had autopsy-proven diagnoses, 27 of whom had SGP documented at some point during their illness. Major diagnoses associated with SGP were: PSP (9 patients), Parkinson disease (PD) (10 patients), multiple system atrophy (2 patients), corticobasal degeneration (2 patients), Creutzfeld-Jakob disease (1 patient) and Huntington disease (1 patient). In none of the diagnostic groups was the age of onset or disease duration significantly different between cases with SGP and those without SGP. In the PD patients, the UPDRS motor score differed significantly between groups (p = 0.01) with the PD/SGP patients having greater motor deficit than those without SGP. Conclusion Although a common feature of PSP, SGP is not diagnostic for this condition and can be associated with other neurodegenerative causes of parkinsonism including PD.

Is Levodopa Response a Valid Indicator of Parkinson's Disease?

“…Although a poor or absent response to levodopa (LD) has previously been one of the clinical diagnostic criteria for PSP, several papers have reported a beneficial response similar to the 44% that we observed. This can potentially lead to an incorrect diagnosis of PD in patients with PSP, particularly in view of our previous observation that supranuclear gaze palsy is not uncommon in people with PD 32 . Lang suggests that the overall response rate to LD in PSP is about 26% 33 .…”

A case of Gerstmann-Straussler-Scheinker (GSS) disease with supranuclear gaze palsy

“…For example, there are reports of familial Creutzfeldt-Jakob disease (CJD) with mutations at prion proteins codons 129 and 200 manifesting with a PSP-like phenotype, while a thalamocortical MM2 subtype can mimic PSP in sporadic CJD cases [7]. In a study analyzing autopsy data of 27 patients with supranuclear gaze palsy and parkinsonism, pathology was consistent with a variety of neurodegenerative diseases in addition to CJD, including PSP, Parkinson disease, corticobasal degeneration, and multiple system atrophy [8]. Patients with ataxia with oculomotor apraxia (AOA) and ataxia-telangectasia can also exhibit supranuclear palsy, though AOA is characterized by more severe impaired pursuit.…”

A Systematic Review and Case Series of Ziprasidone for Psychosis in Parkinson’s Disease

“…All patients were evaluated in the Washington University Movement Disorders Center between 1996 and January 2018 by a movement disorders specialist. This center has used an electronic medical record for over 20 years, Medical Automated Records System (MARS), which permits automated retrospective queries of collected clinical data [19]. A search was performed for patients with a diagnosis of idiopathic Parkinson’s disease OR ICD–9–CM code of 332.0 and a history of ziprasidone in the medication list.…”