Qualitative Analysis of Decision to Pursue Electrical Brain Stimulation by Patients With Drug-Resistant Epilepsy and Their Caregivers.

Background and Objectives: To understand why patients with drug-resistant epilepsy (DRE) pursue invasive electrical brain stimulation (EBS). Methods: We interviewed patients with DRE (n = 20) and their caregivers about their experiences in pursuing EBS approximately 1 year post device implant. Inductive analysis was applied to identify key motivating factors. Results: The cohort included participants aged from teens to 50s with deep brain stimulation, vagus nerve stimulation, responsive neurostimulation, and chronic subthreshold cortical stimulation. Patients' motivations included (1) improved quality of life (2) intolerability of antiseizure medications, (3) desperation, and (4) patient-family dynamics. Both patients and caregivers described a desire to alleviate burdens of the other. Patient apprehensions about EBS focused on invasiveness and the presence of electrodes in the brain. Previous experiences with invasive monitoring and the ability to see hardware in person during clinical visits influenced patients' comfort in proceeding with EBS. Despite realistic expectations for modest and delayed benefits, patients held out hope for an exceptionally positive outcome. Discussion: Our findings describe the motivations and decision-making process for patients with DRE who pursue invasive EBS. Patients balance feelings of desperation, personal goals, frustration with medication side effects, fears about surgery, and potential pressure from concerned caregivers. These factors together with the sense that patients have exhausted therapeutic alternatives may explain the limited decisional ambivalence observed in this cohort. These themes highlight opportunities for epilepsy care teams to support patient decision-making processes.

Which Sport Is More Dangerous: Ice Hockey or Field Hockey? A Review of the Head and Neck Injuries Associated With the Two Sports.

PURPOSE: Ice hockey and field hockey are contact sports with the potential for injury, especially to the head and neck regions. The purpose of this study is to estimate and compare hospital admission (injury severity) between ice hockey and field hockey of those who presented to the emergency department with head and neck injuries. METHODS: The investigators designed and implemented a 20-year retrospective cohort study using the National Electronic Injury Surveillance System database. We included data related to ice hockey and field hockey injuries from January 2000 to December 2019 in this study. The primary predictor variable was sport played (ice hockey vs field hockey). Secondary predictor variables and covariates were derived from patient and injury characteristics. The primary outcome variable was hospital admission. Logistic regression was used to determine independent risk factors for the outcome variable. RESULTS: Our final sample was composed of 5,472 patients: 4,472 patients suffered head and neck injuries from ice hockey while the remaining 1,000 patients suffered head and neck injuries from field hockey. Players less than 18 years old were associated with 2.07-fold odds of admission (P < .01). Injury to the head (odds ratio [OR] = 14.339; 95% confidence interval [CI], 2.0 to 105.1; P < .01) and neck (OR = 89.260; 95% CI, 11.2 to 712.6; P < .01) were independently associated with an increased odds of admission. Relative to contusions/abrasions, players who suffered a concussion (OR = 141.637; 95% CI, 11.5 to 1,741.5; P < .01), fracture (OR = 155.434; 95% CI, 17.0 to 1,419.2; P < .01), internal organ injury (OR = 186.450; 95% CI, 15.5 to 2,236.8; P < .01), or hematoma (OR = 23.046; 95% CI, 1.2 to 442.5; P < .05) were all independently associated with an increased odds of admission. Ice hockey was not an independent risk factor for admission relative to field hockey. CONCLUSIONS: The findings of this study suggest that ice hockey was more associated with injuries to the head and neck as well as with concussions and internal organ injury compared to field hockey. However, ice hockey was not associated with increased risk of hospitalization relative to field hockey.

A Comparison of 2,845 Head and Neck Injuries in Various Martial Arts.

PURPOSE: The primary purpose of this study is to estimate and compare the frequencies, types, and hospital admission rates of head and neck injuries in subjects who practice different martial art fighting styles, including karate, kung fu, kickboxing, taekwondo, judo, and jiu jitsu. METHODS: The investigators designed and implemented a 20-year cross-sectional study using the National Electronic Injury Surveillance System database. Information related to head and neck martial art injuries from January 2000 through December 2019 was included in this study. Study variables were obtained from both patient demographics and injury characteristics (date of injury, diagnosis, body part, type of martial art, and disposition). Patient and injury characteristics were compared through χ2 and independent sample tests. RESULTS: Taekwondo was the most likely type of martial art to lead to a head injury (P < .01), whereas jiu jitsu (P < .01) and judo (P < .01) were most likely to cause neck injuries. The type of martial art was not significantly associated with admission rates (P = .190); however, patients with head injuries were more likely to be admitted relative to patients who did not suffer head injuries (P < .05). Moreover, injuries secondary to judo were more likely to result in admissions relative to that of all other martial arts (P < .05). CONCLUSIONS: Judo was the most severe martial art during this time period as it led to the highest rate of hospital admissions. Karate injuries are most likely to occur in younger populations of fighters under the age of 18 years.

Aneurysmal bone cyst of the head & neck: A review of reported cases in the literature.

The aneurysmal bone cyst (ABC) is a benign, blood-filled bony lesion that notoriously affects the vertebral column and long bones of the body. Rarely, ABCs can manifest in the head and neck (HNABC). The purpose of this paper is to provide a comprehensive review of existing literature on HNABC. This review includes: case reports and case series on ABCs manifesting in the head and neck region published in the English language. All cases identified via PubMed were analyzed individually. Articles were included according to specified eligibility criteria. The total number of cases analyzed were 72 (39 case reports; 33 case series). The average age at the time of HNABC diagnosis was 19.1 years (range: 0.42-62 years). The ratio of prevalence by gender affected exhibited an even 1:1 male-to-female split. CT in combination with MRI proved to be the most prevalent imaging modality utilized (37.2%). HNABC was most commonly detected in the mandible (37.1%), followed by the sinus (14.3%) and cranium (11.4%). The most frequently employed single treatment modality was surgical excision (94.1%). 94.4% of patients were alive with no evidence of disease at follow-up, while 5.6% of patients exhibited disease at follow-up. The average follow-up period was 3.59 years (range: 0.17-17.0 years). Aneurysmal bone cyst of the head and neck region is a rare condition that should be considered in the differential diagnosis of bony, vascular lesions presenting in the aforementioned locations. Since aneurysmal bone cyst of the head and neck region can present as a rapidly growing, expansive and destructive lesion, it is crucial that clinicians are aware of this entity so that patients are accurately diagnosed and treated.

Central giant cell granuloma of the head & neck: A case report and systematic review.

PURPOSE: The purpose of this paper is to describe a recent case of central giant cell granuloma (CGCG) that rapidly progressed post corticosteroid treatment while also providing a review of the existing literature on CGCG of the head and neck (HNCGCG), with particular emphasis on extra-mandibular and maxillary cases. MATERIALS AND METHODS: The investigators designed and implemented a 32-year review of literature, using the online databases: PubMed, Google Scholar, Medline, and Proquest. The total number of cases analyzed was 55 (42 case reports; 3 case series; 8 comparative studies; 1 retrospective cohort). CASE PRESENTATION: We present a case of a CGCG in a 10-year old male. The lesion originated in the right anterior mandibular body and progressed after corticosteroid treatment. Diagnosis was made using a combination of imaging and histology. A timely debulking procedure of the hemi-mandible was performed and there was no recurrence of the lesion at follow up. RESULTS: The average age at the time of diagnosis of CGCG was 27.5 years. HNCGCG was most commonly detected in the jaw (43.1%), but was also found in the temporal bone (33.3%). The most frequently employed treatment modality was complete surgical excision (76.9%). 93.2% of patients were alive with no evidence of disease at follow-up, while 6.8% of patients exhibited recurrence at follow-up. The median follow up was 13 months. CONCLUSION: It is important for clinicians to recognize that CGCGs are capable of manifesting outside of the jaw. CGCG should be considered in the differential diagnosis of non-odontogenic radiolucent lesions, especially in young patients. CGCGs also need to be distinguished from brown tumor of hyperparathyroidism (BTH) and giant cell tumors, which are histologically similar.

What Effect Does Epstein-Barr Virus Have on Extranodal Natural Killer/T-Cell Lymphoma Prognosis? A Review of 153 Reported Cases.

The primary aim of this review is to identify the relationship between Epstein-Barr virus (EBV) and prognosis in extranodal natural killer/T-cell lymphoma (ENKTL). Additionally, a literature review of ENKTL was carried out. The investigators designed and implemented a 21-year literature review using the online databases PubMed and Google Scholar. The total number of cases analyzed was 153 (64 case reports; one comparative study; one systematic review). Information related to ENKTL from July 1999 to February 2021 was included in the study. Study variables included: patient demographics, tumor classification, screening modalities, tumor characteristics, symptomatology, treatment, and prognosis. The average age at diagnosis was 50.9 years (range: 4-90 years). Patients of Asian ethnicity were most commonly affected, and there was a 1.6:1 male to female ratio. ENKTL was most frequently detected in the head and neck region, and 53.1% of cases metastasized. Of all head and neck cases, the nose was the most affected location. Immunohistochemistry positivity included: EBV (32.0%), CD2 (96.6%), CD3ϵ (81.7%), CD43 (91.7%), CD56 (86.4%), Granzyme (97.1%), Perforin (90.9%), TIA-1 (97.8%), p53 (33.3%). The most frequently employed single treatment modality was chemotherapy alone, and 34.2% of patients expired within five years of diagnosis. The average follow-up period was 16.51 months (range: 0.25-66 months). EBV was significantly associated with metastatic ENKTL (χ2 = 4.36; CV = 3.84; p = 0.037). We found no association between EBV and ENKTL prognosis (χ2 = 17.2; CV = 21.0; p = 0.14).

Fertility-Preserving Management of Uterine Arteriovenous Malformation in a 16-Year-Old Female.

Arteriovenous malformations (AVM) are abnormal connections between arteries and veins without a capillary bed, creating high- and low-flow areas that are prone to bleeding. Uterine AVMs can be congenital or acquired with an incidence of 0.1%. Acquired cases are usually caused by uterine instrumentation, trauma, infection, or gestational trophoblastic disease. Patients typically present with sudden onset of heavy vaginal bleeding. Diagnosis is made using angiography, ultrasound, computerized tomography, or magnetic resonance imaging. After patients are stabilized, management depends on their desire for future fertility and may include hysterectomy or endovascular embolization. We present the case of a 16-year-old G1P0010 female with recurrent vaginal bleeding caused by a uterine AVM. To preserve the patient's fertility, a selective embolization approach was employed using microcoils and gel foam. This case highlights a unique treatment option for uterine AVMs in patients who desire fertility preservation. Additionally, we review the diagnostic imaging and treatment options for uterine AVMs.

Horner Syndrome Secondary to Osteochondroma of the First Rib: A Case Report.

Osteochondroma is the most common benign tumor of bone that often produces no symptoms unless the enlarged mass affects nearby structures. Rarely, Horner syndrome can be caused by an osteochondroma. A five-year-old female with a past medical history of seizure-like activity presented to the emergency department on three separate occasions within one month. She exhibited neurological deficits, including miosis and ptosis, resulting in the diagnosis of Horner syndrome. Computerized tomography (CT) demonstrated a calcified and ossified lesion arising from the right first rib and transverse process that was suspicious for an osteochondroma or chondrosarcoma with neuroblastoma lower on the differential diagnosis. Given the patient's escalating clinical symptomatology and suspicious features of the lesion, a CT guided-bone biopsy was performed. Pathology revealed an osteochondroma that was eventually resected by neurologic and orthopedic surgeries. In this case report, we review the sympathetic innervation to the head, eye, and neck, the most common etiologies of Horner syndrome, and elucidate imaging modalities useful for diagnosing osteochondroma. Horner syndrome secondary to osteochondroma of the first rib has been documented only once before.

Novel E815K knock-in mouse model of alternating hemiplegia of childhood.

De novo mutations causing dysfunction of the ATP1A3 gene, which encodes the α3 subunit of Na+/K+-ATPase pump expressed in neurons, result in alternating hemiplegia of childhood (AHC). AHC manifests as paroxysmal episodes of hemiplegia, dystonia, behavioral abnormalities, and seizures. The first aim of this study was to characterize a novel knock-in mouse model (Atp1a3E815K+/-, Matoub, Matb+/-) containing the E815K mutation of the Atp1a3 gene recognized as causing the most severe and second most common phenotype of AHC with increased morbidity and mortality as compared to other mutations. The second aim was to investigate the effects of flunarizine, currently the most effective drug used in AHC, to further validate our model and to help address a question with significant clinical implications that has not been addressed in prior studies. Specifically, many E815K patients have clinical decompensation and catastrophic regression after discontinuing flunarizine therapy; however, it is not known whether this is congruent with the natural course of the disease and is a result of withdrawal from an acute beneficial effect, withdrawal from a long-term protective effect or from a detrimental effect of prior flunarizine exposure. Our behavioral and neurophysiological testing demonstrated that Matb+/- mice express a phenotype that bears a strong resemblance to the E815K phenotype in AHC. In addition, these mice developed spontaneous seizures with high incidence of mortality and required fewer electrical stimulations to reach the kindled state as compared to wild-type littermates. Matb+/- mice treated acutely with flunarizine had reduction in hemiplegic attacks as compared with vehicle-treated mice. After withdrawal of flunarizine, Matb+/- mice that had received flunarizine did neither better nor worse, on behavioral tests, than those who had received vehicle. We conclude that: 1) Our mouse model containing the E815K mutation manifests clinical and neurophysiological features of the most severe form of AHC, 2) Flunarizine demonstrated acute anti-hemiplegic effects but not long-term beneficial or detrimental behavioral effects after it was stopped, and 3) The Matb+/- mouse model can be used to investigate the underlying pathophysiology of ATP1A3 dysfunction and the efficacy of potential treatments for AHC.

Mechanisms of increased hippocampal excitability in the Mashl+/- mouse model of Na+ /K+ -ATPase dysfunction.

OBJECTIVE: Na+ /K+ -ATPase dysfunction, primary (mutation) or secondary (energy crisis, neurodegenerative disease) increases neuronal excitability in the brain. To evaluate the mechanisms underlying such increased excitability we studied mice carrying the D801N mutation, the most common mutation causing human disease, specifically alternating hemiplegia of childhood (AHC) including epilepsy. Because the gene is expressed in all neurons, particularly γ-aminobutyric acid (GABA)ergic interneurons, we hypothesized that the pathophysiology would involve both pyramidal cells and interneurons and that fast-spiking interneurons, which have increased firing rates, would be most vulnerable. METHODS: We performed extracellular recordings, as well as whole-cell patch clamp recordings from pyramidal cells and interneurons, in the CA1 region on hippocampal slices. We also performed immunohistochemistry from hippocampal sections to count CA1 pyramidal cells as well as parvalbumin-positive interneurons. In addition, we performed video-electroencephalography (EEG) recordings from the dorsal hippocampal CA1 region. RESULTS: We observed that juvenile knock-in mice carrying the above mutation reproduce the human phenotype of AHC. We then demonstrated in the CA1 region of these mice the following findings as compared to wild type: (1) Increased number of spikes evoked by electrical stimulation of Schaffer collaterals; (2) equalization by bicuculline of the number of spikes induced by Schaffer collateral stimulation; (3) reduced miniature, spontaneous, and evoked inhibitory postsynaptic currents, but no change in excitatory postsynaptic currents; (4) robust action potential frequency adaptation in response to depolarizing current injection in CA1 fast-spiking interneurons; and (5) no change in the number of pyramidal cells, but reduced number of parvalbumin positive interneurons. SIGNIFICANCE: Our data indicate that, in our genetic model of Atp1α3 mutation, there is increased excitability and marked dysfunction in GABAergic inhibition. This supports the performance of further investigations to determine if selective expression of the mutation in GABAergic and or glutamatergic neurons is necessary and sufficient to result in the behavioral phenotype.