A 78-Year-Old Woman with Sudden Onset of Left-Sided Hemiballismus.

BACKGROUND Hemiballismus is the most severe form of chorea and is a hyperkinetic disorder characterized by involuntary, high-amplitude movements of the ipsilateral arm and leg, due to lesions of the contralateral side of the central nervous system. Ischemic or hemorrhagic strokes and nonketotic hyperglycemia are predominant etiologies of hemiballismus. Case reports highlighting hemiballismus associated with temporal and parietal lobe infarcts have been published, although research of frontal lobe involvement is limited. CASE REPORT A 78-year-old woman presented to the Emergency Department with sudden-onset left-sided hemiballismus. On examination, she was alert, oriented to self and time, and able to follow commands. Her neurologic examination was notable for left-sided hemiballismus, described by the provider as periodic, uncontrolled, and involving a "flinging" motion of the left upper and lower extremities, sparing the face. She was treated with benzodiazepines in the Emergency Department and administered intravenous levetiracetam. Computed tomography of the head without contrast revealed an old left basal ganglia lacunar infarct. The patient was then admitted to the inpatient service, where magnetic resonance imaging of the brain revealed an acute punctate left superior frontal gyrus cortical infarct. Outpatient electroencephalogram revealed right anterior hemisphere dysfunction. CONCLUSIONS We describe a patient with left-sided sudden onset hemiballismus with an acute infarct of the ipsilateral superior frontal gyrus. This case highlights that brain lesions separate from the basal ganglia can induce hemiballismus, particularly within the frontal lobe, which warrants further research into precentral sulcus functioning and its role in modulating motor activity.

A descriptive study of eye and head movements in versive seizures.

BACKGROUND: Versive seizures, consisting of forced, involuntary, sustained and unnatural turning of eyes and head toward one side, lateralize to the hemisphere contralateral to the direction of the eye and head turn. The characteristics of eye and head movements in version have been rarely and incompletely studied in spontaneous epileptic seizures as opposed to direct cortical stimulation studies. METHODS: We performed a single center retrospective analysis of a cohort of 28 patients with 43 seizures, who had been admitted to the adult epilepsy monitoring unit at University Hospitals Cleveland Medical Center between January 2009 and August 2020. We only included patients with clear, high-resolution seizure videos and interpretable EEG. RESULTS: The eye movements were conjugate and contralateral to the hemisphere of seizure onset in 100% of the focal-onset seizures. The eye movements were saccadic in 89.3% with a predominant vector in oblique upward direction in 86.8% of the seizures. Head deviation was present in 100% of the seizures and the eyes and head deviated in the same direction in 97.6% of the seizures. In addition to deviation along the horizontal meridian, there was a vertical component to the head deviation as well, as evidenced by movement of the chin upward along the vertical axis in 93% of the seizures, thus indicating strong activation of the sternocleidomastoid muscle ipsilateral to the hemisphere of seizure onset. Concomitant facial motor activity ipsilateral to the direction of version was seen in 93% of the seizures. The most common pattern was a clonic superimposed on tonic facial contraction. DISCUSSION: Version remains a reliable and highly lateralizing sign. The majority of the eye movements during version occur in a saccadic fashion rather than one smooth movement, mostly in an oblique upward direction. Head deviation is very closely associated with eye deviation, thus indicating a common symptomatogenic zone for both, which is most likely the frontal eye field. A high concurrence of ipsilateral facial motor activity with version is likely because of close proximity of the frontal eye field to the face area in the primary motor cortex.

Nonmydriatic Retinal Photography in the Outpatient Neurology Resident Clinic.

BACKGROUND: The funduscopic examination is an essential component of the neurologic examination. However, examination of the ocular fundus with a direct ophthalmoscope is often difficult. Nonmydriatic ocular fundus photography allows direct visualization of the ocular fundus with high-quality photographs. We used nonmydriatic ocular fundus photography to improve patient care and funduscopy skills of residents in the Neurology Resident Clinic. METHODS: At the time of triage, funduscopic photographs of all new neurology resident clinic patients were taken. The images were imported into the hospital's imaging software. The residents completed a full neurologic examination, including a funduscopic examination with a handheld ophthalmoscope. At the time of staffing the patients with the attendings, the residents received immediate feedback and teaching on retina photograph evaluation. RESULTS: A total of 255 patients were enrolled. Of those, 230 (90%) had at least one high-quality funduscopic photograph. Retinal photographs were normal in 161 (70%). Out of the 69 abnormal photographs, only 7% of abnormalities were detected by the residents. Ninety-three percent of residents found the retinal photographs useful. CONCLUSIONS: Nonmydriatic ocular fundus photography improved the care in patients presenting to a Neurology resident clinic and facilitated residents in recognizing funduscopic findings. Its benefits are clear when one considers (1) the high risk of negative patient outcomes and possible medicolegal consequences due to missed findings, (2) the ease of incorporating retinal photographs into the patients' medical records, and (3) the benefit of improving resident education in regard to the ophthalmologic examination.

Ictal Lid Movements: Blinks and Lid Saccades.

Two types of lid movements, blinks and lid saccades, have discrete kinematic properties and physiology. These differences are reflected in distinct phenomenology of disorders affecting their neural substrate. Proof of this principle was seen in two patients, one with parietal lobe epilepsy and the other with temporal lobe epilepsy. The lid movements in the patient with parietal lobe epilepsy were rhythmic, yoked, and had a rapid upward component that instantaneously followed a slow downward drift. These cyclic movements strikingly resembled nystagmus, but unlike typical eye nystagmus, the rapid upward component was pathological and seemed to involve a saccadic mechanism. We suggest the terms "ictal lid saccades" or "ictal lid nystagmus" to describe such a phenomenon. In contrast, the patient with temporal lobe epilepsy had ipsilateral lid movements with rapid downward trajectories resembling reflex or spontaneous blinks. The term "ictal blink" is appropriate for this phenomenon.

Systematic Literature Review of Psychiatric Comorbidities in Adults with Epilepsy.

BACKGROUND AND PURPOSE: Mental illness is disproportionately common in people with epilepsy (PWE). This systematic literature review identified original research articles that reported the prevalence of psychiatric comorbidities based upon clinical assessments in a sample of PWE and assessed the clinical features of the populations found in studies included in our review of mental health comorbidity. METHODS: The included articles were written in English and published from 2008 to 2018, and focused on adults aged ≥18 years who had psychiatric diagnoses determined in clinical assessments, such as those found in medical records, clinician psychiatric evaluations, structured diagnostic interviews, and mental health screening questionnaires specific for a psychiatric disorder. The primary outcome was the prevalence of psychiatric comorbidities as a percentage of the total sample of PWE. Additional data included the overall sample size, mean age, epilepsy type, study design, and method of diagnosis. A modified Newcastle Ottawa Scale was used to assess the quality of the studies. All 23 articles that were consistent with the inclusion criteria were related to observational studies. RESULTS: Mood disorders and anxiety disorders were the most common psychiatric comorbidities, with prevalence rates of 35.0% and 25.6%, respectively. Major depressive disorder was the most common mood disorder, with a prevalence of 24.2%. Post-traumatic stress disorder (PTSD) had the highest reported prevalence among anxiety disorders, at 14.2%, followed by general anxiety disorder at 11.1%. Other comorbidities included psychosis (5.7%), obsessivecompulsive disorder (3.8%), schizophrenia (1.7%), bipolar disorder (6.2%), and substance abuse (7.9%). The pooled prevalence of suicidality, as reported for two studies, was 9.3%. Temporal lobe epilepsy (TLE) was associated with higher levels of psychiatric comorbidity. Two (8.7%) of the 23 studies compared psychiatric comorbidities in TLE with that of extratemporal lobe epilepsy (ETLE), and one of these two studies found that depression was more common in TLE (53.8%) than in ETLE (25%). Regarding seizure types, partial seizures were associated with a higher prevalence of depression vs generalized seizures. CONCLUSIONS: This systematic literature review of recent original research found a relatively high prevalence of mental health comorbidities in PWE. Mood and anxiety disorders are the most common comorbidities, while psychotic spectrum conditions such as schizophrenia and bipolar disorder are much rarer. The prevalence of comorbidity may vary with the epilepsy type and treatment responsiveness. These findings suggest that screening tools for depression and anxiety should be included as part of the training for epilepsy care, while resources for other relatively common conditions such as PTSD and substance abuse disorders should be readily available to neurology specialists who treat PWE.

Health literacy and education level correlates of participation and outcome in a remotely delivered epilepsy self-management program.

SIGNIFICANCE: Health literacy, the ability to understand necessary health information to make proper health decisions, has been linked to greater frequency of hospitalizations. However, there is limited literature on the associations between health literacy and outcomes in patients with epilepsy, and thus, this secondary analysis investigates the associations between health literacy and outcomes in patients with epilepsy enrolled in the self-management intervention "Self-management for people with epilepsy and a history of negative events" (SMART). We examined the associations between higher health literacy and higher education level and outcomes of the SMART trial. METHODS: This is a secondary analysis of data from the SMART self-management intervention, where individuals were randomized to the SMART intervention or a 6-month waitlist (WL) control. Health literacy was assessed at baseline before randomization using the Rapid Estimate of Adult Literacy in Medicine (REALM-R). Education level was self-reported by participants at baseline. Pearson correlations between REALM-R scores and continuous demographic and clinical variables were conducted. Point-biserial Pearson correlations were computed for REALM-R and dichotomous variables. The effect of education on change in negative health events (NHEs) counts from baseline to six months was conducted using a linear regression. A logistic regression with health literacy and randomization arm as predictors and improvement in NHE (1 = improvement, 0 = no change or increased NHEs at 6 months) as the outcome was conducted. RESULTS: Lower education and lower income were significantly correlated with lower health literacy (p < 0.001 and p = 0.03). Higher education level was associated with a greater improvement in 6-month seizure counts (rs(105) = 0.29, p = 0.002), and a greater improvement in total 6-month NHEs (rs(95) = 0.20, p = 0.045). Health literacy was not associated with change in NHEs or with study retention. CONCLUSIONS: The SMART intervention appears effective for individuals regardless of health literacy competency. Nevertheless, individuals with higher levels of education have fewer epilepsy complications, and thus, those with limited education may still require additional support while participating in epilepsy self-management programs.

Effects of a remotely delivered group-format epilepsy self-management program on adverse health outcomes in vulnerable people with epilepsy: A causal mediation analysis.

BACKGROUND: People with epilepsy frequently experience negative health events (NHEs), such as emergency room visits or hospitalizations for epilepsy-related complications despite significant advances in care. We developed a novel remotely delivered group-format epilepsy self-management program ("Self-management for people with epilepsy and a history of negative health events"; SMART). In a 6-month randomized controlled trial (RCT), SMART participants had significant decreases in NHEs, as well changes in attitudes and behaviors compared to a wait-list (Sajatovic et al., 2018). This secondary analysis from the RCT characterizes the indirect causal effects of SMART on NHE improvements that may be mediated by specific improvements in self-management, self-efficacy, social support, quality of life, and depression symptom severity. METHODS: Participants were adults with epilepsy and a NHE in the prior 6 months. There were 60 participants in each RCT arm (SMART versus wait-list) and assessments were conducted at baseline, 10 weeks and 24 weeks. The outcome was a binary variable measuring NHE improvement at week 24. A counterfactual-based mediation framework was used to determine whether improvements or changes in attitudes and behaviors at specific time points or across the study period, mediated the impact of SMART on NHE improvements. RESULTS: At week 24, SMART contributed to significant improvements in NHEs compared to those in wait-list (odds ratio = 3.2, p = 0.015). SMART was significantly associated with improvements and changes in aspects of self-management, self-efficacy, quality of life, and depression symptom severity at week 10, and significant improvements between baseline and week 24 in overall self-management and quality of life. Mediation analyses demonstrated that a portion of the effect (∼20-30 %; p < 0.05) of SMART on NHE improvement was also indirectly mediated by early improvements in depression symptom severity and quality of life. CONCLUSIONS: This mediation analysis of the SMART intervention demonstrates that in addition to its direct effect on improving NHEs in people with epilepsy, early improvements in depression symptom severity and quality of life indirectly mediated ∼20-30 % of the intervention's effect. These results demonstrate the promise of self-management approaches as a key component of an optimal healthcare model for people with epilepsy, particularly those with a recent history of NHEs.

Tremor in chronic inflammatory demyelinating polyneuropathy: Proof of unifying network model for dystonia.

Traditional hypotheses for the pathogenesis of dystonia, the third most common movement disorder, have focused primarily on the basal ganglia. Contemporary theories have emphasized the role of the cerebellum. The modulation of peripheral proprioception also affects dystonia. We proposed a unifying network model for dystonia where the cerebellum, basal ganglia, and peripheral proprioception are connected in a circuit that forms the neural integrator network, ensuring steady position. We suggested that impairment anywhere along this circuit leads to common phenomenology-slow drifts followed by corrective movements, resembling dystonic tremor. We tested this concept in a patient with chronic inflammatory demyelinating polyneuropathy with resulting abnormal proprioception. Quantitative assessment of tremor in this patient revealed drifts in limb position followed by corrective movements and superimposed sinusoidal oscillations-consistent with neural integrator dysfunction. This unique case of chronic inflammatory demyelinating polyneuropathy describes the role of proprioception on the unifying network model for dystonia.