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We describe a unique appearance of spiral drawings, which could add as another clinical sign for FT. Consider looping of spirals as another diagnostic clue to FT.
The clinical hallmarks of a FT include a variable combination of positive signs, such as inconsistency (variability and distractibility), incongruence with known tremor syndromes, entrainment, and coactivation, although these findings might not always be present. We describe a unique appearance of spiral drawings, which could add as another clinical sign for FT. Consider looping of spirals as another diagnostic clue to FT.
The aim of this study is to assess clinical and pathologic correlations of jaw tremor in 3 cohorts enrolled in a long-term aging study. Jaw/lip tremor has been described in various movement disorders but the impact of seeing a jaw tremor on clinician diagnosis and whether the presence of isolated jaw tremor is correlated with subsequent phenoconversion to a different movement disorder are unclear.
Data from the Arizona Study of Aging and Neurodegenerative Disease, a longitudinal clinicopathologic study, were used. Control subjects (n = 708) did not have any tremor or parkinsonism. At initial evaluation, 276 subjects who had jaw tremor were categorized as isolated jaw tremor (jaw tremor without limb action tremor or parkinsonism), suspect/possible PD (1 or 2 cardinal features of PD without a history of dopaminergic treatment), parkinsonism (probable PD and other parkinsonian disorders), or nonparkinsonian tremor (e.g., essential tremor). Initial clinical diagnosis was compared with "final diagnosis" based rodegenerative diagnosis.
The presence of either jaw tremor in isolation or associated with nonparkinsonian tremor does not portend a neurodegenerative diagnosis.
The Charcot-Marie-Tooth Examination Score (CMTES) has been used since 2005 in clinics to measure impairment in patients with CMT and has provided natural history data for patients with CMT1A, CMT1B, CMTX1, CMT2A, and many other subtypes. Epibrassinolide compound library chemical However, the CMTES requires an in-person visit, and many individuals are unable to travel to CMT centers because of the distance from the clinic or physical disability or more recently because of COVID-19 restrictions. We therefore developed the virtual CMTES (vCMTES) as outlined below. The aim of this study is to create a remote clinical outcome assessment to measure impairment in patients with CMT.
We modified the CMTESv2 replacing the pinprick and vibration items with light touch and position sense, which can be performed remotely by the patient or the patient with an assistant while being observed by the clinic evaluator. Motor evaluations were performed similar to CMTESv2 by the assistant or the patient, while being observed remotely. We developed a standardized protto change and progression in different subtypes. The vCMTES also offers the potential to reach diverse populations that do not have access to CMT centers.
Statistical analyses demonstrated that the vCMTES was reproducible and reliable as a clinical outcome assessment for CMT. Further studies are needed to test responsiveness to change and progression in different subtypes. The vCMTES also offers the potential to reach diverse populations that do not have access to CMT centers.
To examine the verification of a referring hospital's practice of determining death by neurologic criteria (DNC) by an organ procurement organization (OPO) pursuant to the Center for Medicaid and Medicare Services rule §486.344(b).
In this retrospective cohort study, we examined prevalence and factors associated with deviations from acceptable DNC standards, the performance of additional ancillary testing requested by the OPO, resolution of concerns about deviations between referring hospitals and the OPO, and interactions between referring hospitals and the OPO.
The OPO reviewed DNC processes for 645 adult potential organ donors from 64 referral hospitals. Concerns about practice deviations from acceptable standards were identified by the OPO's medical director (also a practicing neurointensivist) on call in 19% (n = 120) and were related to clinical prerequisites (27.2%, n = 49), clinical examination (23.9%, n = 67), and apnea testing (25.3%, n = 97). The top 3 concerns were apnea test results not meetals requested support from the OPO regarding the determination of DNC (10%, n = 64) and declaring physicians were reported to lack knowledge about the institutional DNC policy (4%, n = 23).
Ongoing review of institutional DNC standards and adherence to those standards is an urgent unmet need. Both referring hospitals and OPOs jointly carry responsibility for preventing errors in DNC leading up to organ recovery.
Ongoing review of institutional DNC standards and adherence to those standards is an urgent unmet need. Both referring hospitals and OPOs jointly carry responsibility for preventing errors in DNC leading up to organ recovery.
Social desirability bias, the tendency to underreport undesirable behaviors, may be one reason patients with Parkinson disease (PD) underreport symptoms of impulse control disorders (ICDs).
We compared rates of ICD endorsement on questionnaires administered face-to-face and online in 60 patients with mild-to-moderate idiopathic PD. Participants also completed a self-report measure of social desirability.
We found a significantly higher prevalence of any ICD based on online (56.7%) vs in-person (33.3%) administration. Significantly higher endorsement of items related to hypersexuality in men and compulsive eating and buying in women were found with online administration. Social desirability bias was positively correlated with ICD symptom endorsement across all items and subscales.
The results highlight the importance of social context/setting and the need for sensitivity and discretion when screening for ICD symptoms. Although a higher level of symptom endorsement does not necessarily imply a greater level of accuracy, more work is needed to determine which method of administration is most accurate for clinical and research practice.
The results highlight the importance of social context/setting and the need for sensitivity and discretion when screening for ICD symptoms. Although a higher level of symptom endorsement does not necessarily imply a greater level of accuracy, more work is needed to determine which method of administration is most accurate for clinical and research practice.Neurologic diseases, ranging from Alzheimer dementia to mass lesions in the frontal lobe, may impair decision making. When patients with neurologic disease lack decision-making capacity, but refuse treatment, should they be treated over their objection? To address this type of ethical dilemma in medical illness, Rubin and Prager developed a standardized 7-question approach (1) How imminent is harm without intervention? (2) What is the likely severity of harm without intervention? (3) What are the risks of intervention? (4) What are the logistics of treating over objection? (5) What is the efficacy of the proposed intervention? (6) What is the likely emotional effect of a coerced intervention? (7) What is the patient's reason for refusal? We describe the application of the standardized Rubin/Prager approach as a checklist to the case of a 50-year-old woman with a large frontal lobe meningioma, who lacked capacity as a result of the meningioma, but refused surgery. This approach may be applied to similar ethical dilemmas of treatment over objection in patients lacking capacity as a result of neurologic disease.
We describe the case of a healthy boy diagnosed with reversible cerebral vasoconstriction syndrome (RCVS) and posterior reversible encephalopathy syndrome (PRES).
He was identified after presenting in the emergency department (ED). A review of the 5 previous cases of RCVS complicated with PRES reported in the literature was performed.
A 9-year-old boy was brought to the ED for intense, throbbing headache and vomiting. Physical and neurologic examinations were normal. Brain CT and CSF examination were unremarkable, and he was discharged after symptomatic relief. Five days later, he returned to the ED for generalized tonic-clonic seizures that ceased with levetiracetam. MRI with angiography showed PRES. Systolic hypertension refractory to therapy was documented. New-onset fluctuating right-sided paresis and paresthesia appeared, so MRI was repeated, showing diffuse cerebral vasoconstriction suggesting RCVS. Nimodipine was started with complete resolution of symptoms and normalization of blood pressure. Four weeks after discharge, TD and MRI showed total vasospasm resolution. There was no recurrence in 12-month follow-up.
This case emphasizes the interconnection between RCVS and PRES, highlighting the need to include both as differential diagnoses for severe headache and the essential role of MR angiography in the investigation.
This case emphasizes the interconnection between RCVS and PRES, highlighting the need to include both as differential diagnoses for severe headache and the essential role of MR angiography in the investigation.
Orthostatic hypotension (OH) is the primary manifestation of cardiovascular autonomic dysfunction in Parkinson disease (PD) and can be a prodromal feature of the disease. We review the recent progress in the field of autonomic dysfunction in PD.
Individuals with isolated neurogenic OH should be followed up frequently because they may evolve into PD, dementia with Lewy bodies, or multiple system atrophy. The prevalence of OH in PD increases with disease stages, but the role of levodopa remains unclear. Measurement of supine and standing heart rate and blood pressure allows for accurate identification of neurogenic OH in the clinic.
Accurate identification of neurogenic OH in the clinic is crucial for identification of individuals who may benefit from participation in neuroprotective trials in the future. The treatment of OH in PD should be individualized and may reduce the risk of falls, cognitive impairment, and death.
Accurate identification of neurogenic OH in the clinic is crucial for identification of individuals who may benefit from participation in neuroprotective trials in the future. The treatment of OH in PD should be individualized and may reduce the risk of falls, cognitive impairment, and death.
Novel diagnostic techniques and neurologic biomarkers have greatly expanded clinical indications for CSF studies. CSF is most commonly obtained via lumbar puncture (LP). Although it is generally believed that LPs are well tolerated, there is a lack of supportive data for this claim, and patients anticipate LP to be painful. The objective of this study was to prospectively investigate discordance between patient perception and tolerability of LP.
Adult patients were surveyed before and after LP regarding their perceptions and experience of LP. Physician perceptions were gathered through a web-based survey. Relative risk and Spearman correlation were used to assess the relationship between responses. Paired binomial and paired ordinal responses were compared by McNemar and paired Wilcoxon rank-sum tests.
A total of 178 patients completed the surveys. About half of the patients (58%) reported anxiety pre-LP, at median 3.0 of 10. Physicians overpredicted patients' pre-LP anxiety (median score 5.0,
< 0.