Podemos definir la hemiparesia ipsilateral (HI) como una disfunción de la primera motoneurona que afecta a las extremidades del lado opuesto al esperado, dada la localización de la patología intracraneal desencadenante. La compresión del tracto corticoespinal (tCE) contra el borde libre del tentorio a su paso por el pedúnculo cerebral se conoce como fenómeno de Kernohan-Woltman (FKW). Estudio retrospectivo de pacientes diagnosticados de HI secundaria a un FKW atendidos en nuestra institución, incluyendo un estudio descriptivo de las variables epidemiológicas, clínicas, radiológicas, neurofisiológicas y pronósticas. En el 75% de los casos la clínica fue de instauración aguda o subaguda. El estudio de imagen inicial mostró signos de efecto de masa significativo en la mitad de los pacientes, mientras que la resonancia magnética (RM) permitió identificar una lesión estructural en el pedúnculo cerebral contralateral en dos terceras partes de los casos. En 4 pacientes se verificó una afectación de los potenciales evocados motores (PEM). Durante el seguimiento, 7 pacientes experimentaron una mejoría de la actividad motora, y aproximadamente la mitad de los casos fueron clasificados en los tres primeros grados de la escala de Rankin. En contraste con las series históricas, la mayor parte de nuestros pacientes desarrollaron un FWK a consecuencia de un traumatismo craneoencefálico. La RM es la prueba de imagen de elección tanto para identificar la clásica escotadura del pedúnculo cerebral como para detectar la presencia de una lesión estructural subyacente. El estudio de PEM puede servir de apoyo al diagnóstico, especialmente en casos dudosos. Ipsilateral hemiparesis (IH) can be defined as a paradoxical dysfunction of the first motor neuron involving the extremities on the opposite side to that expected, given the location of the triggering intracranial pathology. Compression of the corticospinal tract (CSt) along its course through the contralateral cerebral peduncle against the free edge of the tentorium, known as the Kernohan-Woltman notch phenomenon (KWNP), represents the main cause of IH. This retrospective study analyzes a series of 12 patients diagnosed with IH secondary to KWNP treated at our institution, including a descriptive study of epidemiological, clinical, radiological, neurophysiological, and prognostic variables. In 75% of the cases, symptoms had an acute or subacute onset. Initial imaging studies showed signs of significant mass effect in half of the patients, whereas magnetic resonance imaging (MRI) identified a structural lesion in the contralateral cerebral peduncle in two thirds of them. Impairment of the motor evoked potentials (MEP) was verified in 4 patients. During follow-up 7 patients experienced improvement in motor activity, and near half of the cases were classified in the first three categories of the modified Rankin scale. In contrast to prior historical series, most of our patients developed a KWNP secondary to a traumatic mechanism. MRI represents the optimal method to identify both the classic cerebral peduncle notch and the underlying structural lesion of the CSt. The use of MEP can help to establish the diagnosis, especially in those cases lacking definite radiological findings.
Background The identification of immunoglobulin G antibodies against the aquaporin-4 channel (AQP-IgG) in the majority of adult patients differentiates neuromyelitis optica as a distinct disease entity. The high specificity of AQP4-IgG for neuromyelitis optica has allowed the identification of seropositive patients with atypical presentations of this disease. Neuromyelitis optica spectrum disorder has been increasingly recognized in children who demonstrate patterns of clinical involvement beyond the traditional boundaries of the optic pathways and spinal cord. Methods This is a single-center, retrospective review comparing demographic, clinical/paraclinical, and laboratory features of children and adults with a serologically confirmed diagnosis of AQP4-IgG-positive neuromyelitis optica spectrum disorder. Results Of 151 reviewed patient charts, 12 pediatric-onset and 31 adult-onset patients had AQP4-IgG-positive neuromyelitis optica spectrum disorder. The mean age of pediatric-onset neuromyelitis optica spectrum disorder was 12 ± 3.58 years with a female predilection (3:1). Pediatric patients showed more frequent involvement of the brainstem (6/12 [50%]); P = .008) and diencephalon (3/12 [25%]; P = .018). A preceding infection was identifiable in only 3 of 12 (25%) pediatric-onset patients. Moreover, disability as calculated on the expanded disability status scale was less severe in pediatric-onset cases compared to adult-onset cases in their most recent assessment (0 [0-9]) vs 6.5 [0-10]; P = .005). Pediatric-onset patients were also more likely to respond to treatment of acute episodes with corticosteroids ± intravenous immunoglobulin and/or plasmapheresis (Clinical Global Impression–Change scale: 2.5 [1-4] vs 4 [1-6], P = .009). Interpretation This retrospective study was able to compare and contrast pediatric- and adult-onset neuromyelitis optica spectrum disorder. Relative to their adult counterparts, pediatric-onset neuromyelitis optica spectrum disorder patients were more likely to respond to treatment and less likely to be disabled from their disease at follow-up. Therefore, pediatric-onset disease may represent a less virulent form of neuromyelitis optica spectrum disorder.
Ipsilateral hemiparesis (IH) can be defined as a paradoxical dysfunction of the first motor neuron involving the extremities on the opposite side to that expected, given the location of the triggering intracranial pathology. Compression of the corticospinal tract (CSt) along its course through the contralateral cerebral peduncle against the free edge of the tentorium, known as the Kernohan-Woltman notch phenomenon (KWNP), represents the main cause of IH. This retrospective study analyses a series of 12 patients diagnosed with IH secondary to KWNP treated at our institution, including a descriptive study of epidemiological, clinical, radiological, neurophysiological, and prognostic variables. In 75% of the cases, symptoms had an acute or subacute onset. Initial imaging studies showed signs of significant mass effect in half of the patients, whereas magnetic resonance imaging (MRI) identified a structural lesion in the contralateral cerebral peduncle in two thirds of them. Impairment of the motor evoked potentials (MEP) was verified in 4 patients. During follow-up 7 patients experienced improvement in motor activity, and near half of the cases were classified in the first three categories of the modified Rankin scale. In contrast to prior historical series, most of our patients developed a KWNP secondary to a traumatic mechanism. MRI represents the optimal method to identify both the classic cerebral peduncle notch and the underlying structural lesion of the CSt. The use of MEP can help to establish the diagnosis, especially in those cases lacking definite radiological findings. Podemos definir la hemiparesia ipsilateral (HI) como una disfunción de la primera motoneurona que afecta a las extremidades del lado opuesto al esperado, dada la localización de la patología intracraneal desencadenante. La compresión del tracto córtico-espinal (tCE) contra el borde libre del tentorio a su paso por el pedúnculo cerebral se conoce como fenómeno de Kernohan-Woltman (FKW). Estudio retrospectivo de pacientes diagnosticados de HI secundaria a un FKW atendidos en nuestra institución, incluyendo un estudio descriptivo de las variables epidemiológicas, clínicas, radiológicas, neurofisiológicas y pronósticas. En un 75% de los casos la clínica fue de instauración aguda o subaguda. El estudio de imagen inicial mostró signos de efecto de masa significativo en la mitad de los pacientes, mientras que la resonancia magnética (RM) permitió identificar una lesión estructural en el pedúnculo cerebral contralateral en dos terceras partes de los casos. En 4 pacientes se verificó una afectación de los potenciales evocados motores (PEM). Durante el seguimiento, 7 pacientes experimentaron una mejoría de la actividad motora, y aproximadamente la mitad de los casos fueron clasificados en los tres primeros grados de la escala de Rankin. En contraste con las series históricas, la mayor parte de nuestros pacientes desarrollaron un FWK a consecuencia de un traumatismo cráneo-encefálico. La RM es la prueba de imagen de elección tanto para identificar la clásica escotadura del pedúnculo cerebral como detectar la presencia de una lesión estructural subyacente. El estudio de PEM puede servir de apoyo al diagnóstico, especialmente en casos dudosos.