Obstructive sleep apnea induced bilateral tonic- clonic seizure of unknown origin: A case series of a novel association.

Background: Obstructive sleep apnea is a highly prevalent disorder, characterized by recurrent events of upper airway obstruction during sleep and associated with recurrent cycles of desaturation and re-oxygenation, sympathetic hyperactivity, and intra-thoracic pressure fluctuations, resulting in fragmentation of sleep and subsequent daytime fatigue with excessive sleepiness. Obstructive sleep apnea-induced bilateral tonic-clonic seizures are unheard of. We aimed to report 3 patients with previously undiagnosed obstructive sleep apnea who presented to the emergency department with new onset bilateral tonic-clonic seizure without any evidential neurological or metabolic cause. Methods: Patient data were obtained from medical records from the Department of Internal Medicine, IPGMER and SSKM Hospital, Kolkata, and Belle Vue Clinic, Kolkata, India. Results: Three male patients (67, 58, and 44 years old) presented with bilateral tonic-clonic seizure disorder without any underlying cause of seizures after rigorous investigations except for moderate to severe obstructive sleep apnea on polysomnography. All 2 patients were seizure-free after being treated with levetiracetam, chronic continuous positive airway pressure therapy in 2, and only continuous positive airway pressure in the other. The patients remained seizure-free on continuous positive airway pressure, even when levetiracetam was withdrawn, suggesting obstructive sleep apnea's causality in their new-onset acute seizures. Conclusion: Although further investigation is required to clarify this association, underlying obstructive sleep apnea should be ruled out in patients with a first-ever bilateral tonic-clonic seizure. Whether or not continuous positive airway pressure alone could effectively treat hypoxia and deranged cortical excitability, which may lead to seizures in cases with longstanding obstructive sleep apnea, is yet to be explored.

Introducción: La apnea obstructiva del sueño es una enfermedad con una alta prevalencia que se caracteriza por episodios recurrentes de obstrucción de las vías respiratorias altas durante el sueño, lo que conlleva ciclos repetidos de hipoxia y reoxigenación, hiperactividad simpática y fluctuaciones en la presión intratorácica. Todos estos procesos dan lugar a una fragmentación del sueño, lo que provoca fatiga diurna y somnolencia excesiva. Las crisis tónico-clónicas bilaterales inducidas por apnea obstructiva del sueño son poco conocidas. Presentamos los casos de tres pacientes con apnea obstructiva del sueño sin diagnosticar previamente que acudieron a urgencias por crisis tónico-clónicas de nueva aparición sin causa neurológica o metabólica aparente. Métodos: Los datos de nuestros pacientes se recogieron de los historiales médicos del servicio de Medicina Interna del Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial Hospital y de la Belle Vue Clinic, ambos en Kolkata (India). Resultados: Tres pacientes varones de 67, 58 y 44 años de edad presentaron convulsiones tónico-clónicas bilaterales sin causa identificada tras examen riguroso, exceptuando una apnea obstructiva del sueño de gravedad moderada a grave observada en la polisomnografía. Los tres pacientes recibieron tratamiento con levetiracetam durante el ingreso; al alta, se pautó tratamiento crónico con presión positiva continua de las vías respiratorias más levetiracetam en dos pacientes, y en el tercero solo presión positiva continua de las vías respiratorias. Ninguno presentó nuevas crisis tras la retirada de levetiracetam, lo que sugiere que la causa de las convulsiones era la apnea obstructiva del sueño. Conclusión: Aunque es necesario realizar más estudios para aclarar esta asociación, debemos descartar la apnea obstructiva del sueño en pacientes con crisis tónico-clónicas bilaterales de nueva aparición. Queda aún por determinar si la presión positiva continua podría tratar de forma efectiva la hipoxia y las alteraciones en la excitabilidad cortical, que podrían provocar crisis en casos de apnea obstructiva del sueño de larga evolución.

Trastorno de la marcha del nivel superior como forma de presentación de una parálisis supranuclear progresiva: descripción de un vídeo caso / Higher-level gait disorder as a presenting manifestation of progressive supranuclear palsy: a video case report

Introducción: El trastorno de la marcha frontal/apraxia de la marcha es un déficit motor del nivel superior con diversas causas, caracterizado por dificultades en el inicio de la marcha (congelación). Nuestro objetivo es presentar una paciente con un trastorno de la marcha del nivel superior con episodios de caídas como manifestaciones iniciales de una parálisis supranuclear progresiva (PSP). Sus datos se obtuvieron de los registros médicos del Servicio de Medicina General del Burdwan Medical College & Hospital (Burdwan, Bengala Occidental, India). Caso clínico: Mujer de 58 años sana que consultó por un trastorno de la marcha con caídas. La exploración neurológica mostró una apariencia facial característica (mirada fija, ojos muy abiertos, ceño fruncido y expresión fija hemifacial inferior), e hipocinesia-rigidez simétrica de predominio axial (postura retrocólica del tronco y el cuello). La exploración de la marcha reveló un trastorno de la marcha del nivel superior, caracterizado por una significativa vacilación inicial, que precisaba ayuda de objetos/personas cercanos. Al iniciar la marcha, los pasos mejoraban relativamente, pero reaparecía una deambulación inefectiva al girar. Presentaba zancadas cortas, congelación, base amplia de sustentación, desequilibrio, movimiento lento de las piernas, arrastre de los pies, y pérdida de la cadencia normal del tronco y las extremidades. Los reflejos posturales estaban alterados. La resonancia magnética cerebral desveló atrofia mesencefálica, dilatación de acueducto de Silvio y III ventrículo, atrofia frontal bilateral y el signo típico del colibrí. Finalmente, la paciente fue diagnosticada de una PSP probable. Conclusiones: Varias etiologías, incluida la PSP, deben considerarse, en el contexto clínico apropiado, si la exploración de la deambulación demuestra un trastorno de la marcha del nivel superior.(AU)

Introduction: In the evaluation of drug-resistant epilepsy, a detailed analysis of the semiology is essential to establish a diagnostic hypothesis of the location of the epileptogenic zone. Cross-sign (CS) is a very infrequent complex manual automatism described for the first time in 2008 and rarely reported in the literature. Case report: We present two cases from our series of patients monitored by video-EEG, one of whom also studied with deep electrodes, in which we describe the location of the discharge while performing the CS. A bibliographic review is also carried out to try to establish a localizing and/or lateralizing value of this sign. Conclusion: The sign of the cross is a rare ictal automatism that occurs in patients with temporal lobe epilepsy. The hand used to make the sign of the cross is the dominant one, regardless of the origin of the crises.(AU)

Higher-level gait disorder as a presenting manifestation of progressive supranuclear palsy: a video case report. / Trastorno de la marcha del nivel superior como forma de presentación de una parálisis supranuclear progresiva: descripción de un vídeo caso.

INTRODUCTION: Frontal gait disorder/gait apraxia is a higher-order motor deficit with various causes, characterized by difficulties with gait initiation, such as freezing or ignition failure. We aimed to report a patient who presented with progressive higher-level gait disorder and fall episodes as the initial manifestations of progressive supranuclear palsy (PSP). Patient data were obtained from medical records from the Department of General Medicine, Burdwan Medical College and Hospital (Burdwan, West Bengal, India). CASE REPORT: A 58-year-old previously healthy woman presented with a gait disorder and fall episodes. Detailed neurological examination highlighted characteristic facial appearance (wide-eyed staring, furrowing of the forehead with a frowning expression, and fixed expression of the lower face). She was hypokinetic-rigid with symmetrical signs and predominant axial rigidity with retrocolic trunk and neck posture. Gait examination revealed a higher-level gait pattern characterized by an exhibition of profound start hesitation requiring assistance from nearby objects/persons. Once walking was underway, steps became relatively better, but ineffective gait re-emerged when she attempted turning. She had short strides, freezing, broad stance base, disequilibrium, slow leg movement, shuffling, and loss of normal fluidity of trunk and limbs. Postural reflexes were impaired. Brain magnetic resonance imaging revealed atrophy of the midbrain, dilated aqueduct of Sylvius and third ventricle, atrophy of frontal lobes and typical hummingbird sign. Diagnosis of probable PSP was finally made. CONCLUSIONS: Several etiologies, including PSP, should be considered in appropriate clinical contexts if gait examination demonstrates a higher-order gait disorder.

TITLE: Trastorno de la marcha del nivel superior como forma de presentación de una parálisis supranuclear progresiva: descripción de un vídeo caso.Introducción. El trastorno de la marcha frontal/apraxia de la marcha es un déficit motor del nivel superior con diversas causas, caracterizado por dificultades en el inicio de la marcha (congelación). Nuestro objetivo es presentar una paciente con un trastorno de la marcha del nivel superior con episodios de caídas como manifestaciones iniciales de una parálisis supranuclear progresiva (PSP). Sus datos se obtuvieron de los registros médicos del Servicio de Medicina General del Burdwan Medical College and Hospital (Burdwan, Bengala Occidental, India). Caso clínico. Mujer de 58 años sana que consultó por un trastorno de la marcha con caídas. La exploración neurológica mostró una apariencia facial característica (mirada fija, ojos muy abiertos, ceño fruncido y expresión fija hemifacial inferior), e hipocinesia-rigidez simétrica de predominio axial (postura retrocólica del tronco y el cuello). La exploración de la marcha reveló un trastorno de la marcha del nivel superior, caracterizado por una significativa vacilación inicial, que precisaba ayuda de objetos/personas cercanos. Al iniciar la marcha, los pasos mejoraban relativamente, pero reaparecía una deambulación inefectiva al girar. Presentaba zancadas cortas, congelación, base amplia de sustentación, desequilibrio, movimiento lento de las piernas, arrastre de los pies, y pérdida de la cadencia normal del tronco y las extremidades. Los reflejos posturales estaban alterados. La resonancia magnética cerebral desveló atrofia mesencefálica, dilatación de acueducto de Silvio y III ventrículo, atrofia frontal bilateral y el signo típico del colibrí. Finalmente, la paciente fue diagnosticada de una PSP probable. Conclusiones. Varias etiologías, incluida la PSP, deben considerarse, en el contexto clínico apropiado, si la exploración de la deambulación demuestra un trastorno de la marcha del nivel superior.

Health-related quality of life and perceived stress of informal caregivers of children and adolescents with intellectual disabilities and ADHD.

Introduction: Informal caregivers of children and adolescents with intellectual disabilities and attention deficit/hyperactivity disorder (ADHD) face numerous challenges. However, no study has yet compared the HRQoL of the caregivers of children and adolescents with these two conditions. We aimed to compare the HRQoL and perceived stress of caregivers of children and adolescents with intellectual disabilities and ADHD. Methods: The HRQoL and perceived stress of informal caregivers of children and adolescents with intellectual disabilities and ADHD (40 in each group) were compared using the perceived stress scale and the Quality of Life Enjoyment and Satisfaction Questionnaire - Short Form, respectively. Results: HRQoL was significantly worse in most dimensions in caregivers of children and adolescents with severe ADHD than in caregivers of children and adolescents with severe intellectual disabilities. However, perceived stress was similar. Conclusion: Differences in the impact of intellectual disability and ADHD on family members' HRQoL should be considered while developing educational programs for patients and their families.

Impact of a specific consultation for patients with progressive forms of multiple sclerosis on the response to their unmet care needs: a cross-sectional study.

BACKGROUND: As their disease evolves, most patients with progressive forms of multiple sclerosis (MS) develop particular healthcare needs that are not always addressed with usual follow-up. To adapt neurological care to these patients, we created a specific consultation for patients with progressive MS in our centre in 2019. OBJECTIVES: To explore the main unmet care needs of patients with progressive MS in our setting, and to establish the usefulness of the specific consultation to address them. METHODS: Literature review and interviews with patients and healthcare professionals were conducted to identify the main unmet needs in routine follow-up. Two questionnaires were developed, assessing the importance of the unmet needs identified and the usefulness of the consultation to meet them, for patients under follow-up in the specific consultation and their informal caregivers. RESULTS: Forty-one patients and nineteen informal caregivers participated. The most important unmet needs were the information about the disease, access to social services and coordination between specialists. A positive correlation was found between the importance of these unmet needs and the responsiveness to each of them in the specific consultation. CONCLUSIONS: The creation of a specific consultation may improve attention to the healthcare needs of patients with progressive MS.

[The electroencephalogram: a complementary test that should not be forgotten to perform before a first seizure]. / El electroencefalograma: una prueba complementaria que no debe olvidarse realizar ante una primera crisis epiléptica.

TITLE: El electroencefalograma: una prueba complementaria que no debe olvidarse realizar ante una primera crisis epiléptica.

[Alcohol as a rescue treatment for essential tremor: too risky to recommend]. / El alcohol como tratamiento de rescate para el temblor esencial: demasiado arriesgado para recomendarlo.

TITLE: El alcohol como tratamiento de rescate para el temblor esencial: demasiado arriesgado para recomendarlo.