Exoftalmos y dolor ocular como presentación clínica de linfoma de Hodgkin con afectación intracraneal al diagnóstico / Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis

La afectación intracraneal del linfoma de Hodgkin (LH) es extremadamente rara, especialmente como forma de presentación de la enfermedad. Muestra un patrón radiológico inespecífico, pudiendo ser confundido con otras entidades de mayor frecuencia y pronóstico radicalmente distinto. Anatomopatológicamente se caracteriza por la presencia de células grandes binucleadas (células de Reed-Sternberg) eIntracranial involvement in Hodgkin's lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network.

In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presentation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis (AU)mbebidas en un entramado inflamatorio. Presentamos el caso de una paciente con dolor ocular y exoftalmos izquierdo como presentación clínica de afectación intracraneal por LH al diagnóstico de su enfermedad y revisamos la literatura más reciente al respecto. En pacientes con LH intracraneal es necesario realizar un estudio de extensión con tomografía computarizada corporal, biopsia de médula ósea y examen oftalmológico. Se asocia con gran frecuencia a enfermedad extracraneal, que marca el pronóstico. La lesión intracraneal presenta buena respuesta al tratamiento, que no sigue un esquema estandarizado. El papel de la cirugía es la realización de una biopsia para confirmar el diagnóstico (AU)

Exophthalmos and ocular pain as clinical debut of intracranial Hodgkin's lymphoma at diagnosis.

Intracranial involvement in Hodgkin's Lymphoma (HL) is extremely unusual, especially at the time of diagnosis. Because of its non-specific radiological behaviour, it can be confused with more common entities with a radically different prognosis. Pathologically, large and bi-nucleated cells, called Reed-Sternberg cells, embedded in an inflammatory network. In this report we describe the clinical case of a patient, with no medical history, with left ocular pain and exophthalmos as presetation of intracranial HL at diagnosis and review the most current literature. Intracranial involvement is often associated with extracranial disease. Therefore, a systemic study including body computed tomography, bone marrow biopsy and ophthalmological evaluation is necessary. Intracranial lesions respond favourably to treatment and the prognosis depends on the extracranial involvement. To date, there is no standardised management scheme for these patients. For us, the primary role of surgery in this context is to perform a biopsy to confirm the histological diagnosis.

Kernohan-Woltman notch phenomenon: an exceptional neurological picture?

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.