Beyond uncal herniation: An updated diagnostic reappraisal of ipsilateral hemiparesis and the Kernohan-Woltman notch phenomenon.

PURPOSE: This works comprehensively analyses a modern cohort of patients with ipsilateral hemiparesis (IH) and discusses the pathophysiological theories elaborated to explain this paradoxical neurological sign according to the findings from contemporary neuroimaging and neurophysiological techniques. METHODS: A descriptive analysis of the epidemiological, clinical, neuroradiological, neurophysiological, and outcome data in a series of 102 case reports of IH published on since the introduction of CT/MRI diagnostic methods (years 1977-2021) was performed. RESULTS: IH mostly developed acutely (75.8%) after traumatic brain injury (50%), as a consequence of the encephalic distortions exerted by an intracranial haemorrhage eventually causing contralateral peduncle compression. Sixty-one patients developed a structural lesion involving the contralateral cerebral peduncle (SLCP) demonstrated by modern imaging tools. This SLCP showed certain variability in its morphology and topography, but it seems pathologically consistent with the lesion originally described in 1929 by Kernohan & Woltman. The study of motor evoked potentials was seldom employed for the diagnosis of IH. Most patients underwent surgical decompression, and a 69.1% experienced some improvement of the motor deficit. CONCLUSIONS: Modern diagnostic methods support that most cases in the present series developed IH following the KWNP model. The SLCP is presumably the consequence of either compression or contusion of the cerebral peduncle against the tentorial border, although focal arterial ischemia may also play a contributing role. Some improvement of the motor deficit should be expected even in the presence of a SLCP, provided the axons of the CST were not completely severed.

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.

Postoperative management of patients with pituitary tumors submitted to pituitary surgery. Experience of a Spanish Pituitary Tumor Center of Excellence.

There is a lack of evidence on timing, frequency, and duration of postoperative endocrine, radiologic, and ophthalmologic assessments that should be performed after pituitary surgery (PS). However, it is known that careful optimization of treatment and follow-up strategies as well as a multidisciplinary approach may have a significant impact on long-term outcomes, improving surgical results, minimize complications and facilitate their correct treatment if occurring, and optimize the hormonal, ophthalmological, and radiological reassessment throughout the follow-up. Considering that there are no specific guidelines on the postoperative management of patients with pituitary tumors (PT), we present our protocol for the postoperative management of patients with PT. It has been elaborated by the multidisciplinary team of a Spanish Pituitary Tumor Center of Excellence (PTCE) that includes at least one neurosurgeon, ENT, neuroradiologist, neuro-ophthalmologist, endocrine pathologist and endocrinologist specialized in pituitary diseases. We elaborated this guideline with the aim of sharing our experience with other centers involved in the management of PT thereby facilitating the postoperative management of patients submitted to PS.

Spinal extradural angiolipomas: 7 new cases and review of the literature. / Angiolipomas espinales epidurales: presentación de 7 casos y revisión de la literatura.

INTRODUCTION: We present our experience with the diagnosis and treatment of spinal angiolipomas (SAL). METHODS: We used our database of surgical patients to gather epidemiological, clinical, diagnostic, treatment, and follow-up data from confirmed cases of SAL. Patients' functional status was evaluated using the modified Japanese Orthopaedic Association (mJOA) score for thoracic myelopathy. RESULTS: We included 7 patients with ages ranging between 42 and 63 years; 4 were women. Lesions in all patients were located in the thoracic epidural space; in 2 cases, lesions extended outside the spinal canal. The most frequent form of presentation was pain combined with progressive myelopathy; patients scored 5 to 9 points on the mJOA scale. Radiological findings varied; this variability clearly depended on the proportion of tumour components (cellular variant in one case, predominantly angiomatous in another, and myxoid in yet another case). Four cases displayed radiological signs of bone involvement. Surgery resulted in excellent mJOA scores except in one case (mJOA score of 10); no recurrences were detected during follow-up (periods ranging from 1 to 5 years). CONCLUSIONS: Extradural SAL can present with highly variable radiological and clinical features. Surgery must be tailored to each individual situation according to tumour extension; in some cases, it may include spinal reconstruction. Surgery results in excellent outcomes regardless of preoperative functional status and it is associated with low recurrence rates, even in case of incomplete resection.

Clinical features, diagnosis and therapy of pituicytoma: an update.

BACKGROUND: Pituicytomas (PTs) are extremely rare, low-grade glial tumors closely related to the neurohypophyseal axis. Definite conclusions concerning the optimal diagnostic and therapeutic approach to these neoplasms are lacking to date, as most of this information has been presented as case reports. METHODS: Retrospective review of case reports published in the scientific literature to date, including a new illustrative example treated in our department. RESULTS: 116 cases were collected. PTs had a higher prevalence in the fifth and sixth decades of life, with a slight male predominance. Main symptoms, which tended to be progressive, included visual field defects and pituitary-hypothalamic dysfunction. Radiologically, PTs were found anywhere along the hypothalamic-pituitary axis mimicking other, more frequent tumors growing in this anatomical region. Surgical treatment included both transcranial or transsphenoidal approaches, and resulted in gross total resection and morbidity rates of 46.8 and 59%, respectively; the latter essentially consisted in anterior and posterior pituitary dysfunction, with limited impact on daily quality of life. CONCLUSIONS: Due to both low frequency and the absence of pathognomonic clinical and/or radiological features, formulating a suspicion diagnosis of PT represents a considerable challenge even for experienced professionals. The indication for treatment should be made on an individual basis, but it is inescapable in the presence of a visual field defect. The surgical approach has to be tailored according to the topography of the tumor and preoperative symptoms; the greatest challenges in accomplishing a gross total removal are represented by the degree of adherence and vascularization of the PT.

Continued declining incidence and improved survival of progressive multifocal leukoencephalopathy in HIV/AIDS patients in the current era.

To evaluate the situation and perspectives of progressive multifocal leukoencephalopathy (PML) in human immunodeficiency virus (HIV)-infected patients, we investigated changes in the incidence, causes, and long-term outcome of this disease in 72 acquired immunodeficiency syndrome (AIDS) patients who were diagnosed with PML from 1996 to 2011. Patients were classified according to the date of diagnosis in the first (1996-2000, n = 35), second (2001-2006, n = 26), and recent or third highly active antiretroviral therapy (HAART) period (2007-2011, n = 11). Overall, the incidence of PML decreased from 14.8 cases/1,000 patients/year in 1996 to 2.6 in 2005 and 0.8 in 2011, and nearly two-thirds of recent cases (64 %) were observed in HIV patients not attending clinical visits. The baseline median CD4+ count was higher in recently PML-diagnosed patients (77 vs. 86 vs. 101 cells/mm(3); p < 0.01), and this fact was associated with a cerebrospinal fluid (CSF) inflammatory profile (from 11 to 31 to 55 %, p = 0.007) and with a significantly longer survival (attributable death, 54 vs. 35 vs. 36 %, respectively, p < 0.01). Thus, the overall 1-year and 3-year survival rates were 55 and 50 %, respectively, increasing to 79 % at 1 year for patients with CD4+ count above 100 cells/mm(3) at diagnosis. In a Cox regression analysis, an older age (hazard ratio, HR 0.76), a baseline CD4+ count above 100 cells/mm(3) (HR 0.33), and a CSF inflammatory profile (HR 0.12) were significantly associated with a longer survival. The clinical presentation and outcome of PML in AIDS patients continue to change dramatically. Now, a declining incidence and long-term survival is observed.

Limbic encephalitis: a clinical-radiological comparison between herpetic and autoimmune etiologies.

BACKGROUND: Herpetic (HE) and autoimmune (AE) encephalitis share clinical and radiological features. We compared both types of encephalitis with the aim of making a differential clinical-radiological pattern. MATERIALS AND METHODS: All cases with a clinical diagnosis of encephalitis who attended our hospital between 1999 and 2012 were reviewed. We selected those cases with positive polymerase chain reaction for herpes simplex virus 1 (HSV-1) in the cerebrospinal fluid (CSF), and those with antineuronal antibodies or paraneoplastic etiology. We compared epidemiological, clinical, CSF, electroencephalographic and radiological findings. RESULTS: Twelve patients with positive polymerase chain reaction for HSV-1, and 10 patients with antineuronal antibody or paraneoplastic etiology were found. The only features found exclusively in one group were the presence of psychiatric symptoms and tumors in AE. Acute onset of symptoms, fever and aphasia were more frequent in HE, which showed higher level of proteins and erythrocyte count in CSF. Neuroimaging was abnormal in all cases of HE, but only in 60% of AE. Insular and diffuse temporal lobe involvement and absence of basal ganglia involvement were more frequent in HE, and mesial temporal involvement in AE. The highest diagnostic values for differentiating HE from AE were the association of acute onset of symptoms and fever (sensitivity 0.92, specificity 1), and the absence of basal ganglia involvement (sensitivity 0.82, specificity 1). CONCLUSIONS: There are few differences between HE and AE. Psychiatric symptoms and association with tumors were unique for AE. Acute onset with fever and absence of basal ganglia involvement in magnetic resonance imaging support a diagnosis of HE.

Infundíbulo-neurohipofisitis: evolución a 10 años de un caso en la edad pediátrica / Infundibulo-hypophysitis in a girl: 10 years follow-up

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[Multimodal CT in the diagnostic workup of stroke]. / TC multimodal en el diagnóstico del código ictus.

Stroke results in significant morbidity and mortality. Recent years have seen a revolution in the diagnosis and treatment of stroke, with advances in diagnostic imaging and improvements in early and specific treatment. Multimodal CT (unenhanced CT, perfusion CT, and CT angiography) is widely available, fast, and efficacious, all of which give it a key role in the early diagnosis of stroke and in the selection of patients who will benefit from thrombolytic treatment. Unenhanced CT is useful for ruling out hemorrhage or lesions that simulate stroke and for evaluating the presence of early signs. Perfusion CT enables us to know the presence and extension of infarcted ischemic tissue (irrecoverable) and of penumbra (tissue at risk that is potentially recoverable), thus ensuring more appropriate selection of candidates for treatment. Finally, CT angiography makes it possible to evaluate the intra- and extra-cranial circulation, to know the vascular alteration that originated the stroke, and to guide treatment (intra-arterial or mechanical thrombolysis).

TC multimodal en el diagnóstico del código ictus / Multimodal CT in the diagnostic workup of stroke

La importancia del ictus y los avances en el tratamiento precoz y específico en los estudios de imagen han contribuido a la revolución diagnóstica y terapéutica del mismo. La TC multimodal (TC basal, TC de perfusión y TC-angiografía) está considerada como una técnica eficaz, rápida y disponible. Resulta clave para el diagnóstico precoz y para la selección de los pacientes que se beneficiarán del tratamiento trombolítico. La TC basal, sin contraste, es adecuada para descartar un origen hemorrágico o una lesión simuladora de ictus y para valorar la existencia de signos precoces. La TC de perfusión nos permitirá conocer la presencia y extensión de tejido isquémico infartado (no recuperable) y de tejido en riesgo o zona de penumbra (potencialmente recuperable), se pudiendo seleccionar así más apropiadamente los pacientes candidatos a tratamiento. Por último, la angio-TC permitirá valorar la circulación intra y extracraneal, conocer la alteración vascular que origina el cuadro y podrá servir de guía para el tratamiento (trombólisis intraarterial o mecánica) (AU)

Stroke results in significant morbidity and mortality. Recent years have seen a revolution in the diagnosis and treatment of stroke, with advances in diagnostic imaging and improvements in early and specific treatment. Multimodal CT (unenhanced CT, perfusion CT, and CT angiography) is widely available, fast, and efficacious, all of which give it a key role in the early diagnosis of stroke and in the selection of patients who will benefit from thrombolytic treatment. Unenhanced CT is useful for ruling out hemorrhage or lesions that simulate stroke and for evaluating the presence of early signs. Perfusion CT enables us to know the presence and extension of infarcted ischemic tissue (irrecoverable) and of penumbra (tissue at risk that is potentially recoverable), thus ensuring more appropriate selection of candidates for treatment. Finally, CT angiography makes it possible to evaluate the intra- and extra-cranial circulation, to know the vascular alteration that originated the stroke, and to guide treatment (intra-arterial or mechanical thrombolysis) (AU)

Anastomosis carotidovertebrobasilar persistente: arteria hipoglosa / No disponible

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Síndrome de leucoencefalopatía posterior en relación con angiopatía amiloide / Posterior leukoencephalopathy syndrome associated with amyloid angiopathy

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Neuropatía del tercer par craneal secundaria al síndrome de hipotensión intracraneal / Third cranial nerve palsy due to intracranial hypotension syndrome

Introducción. El síndrome de hipotensión intracraneal(SHIC) se caracteriza por cefalea ortostática secundaria ahipovolemia de líquido cefalorraquídeo (LCR). Es frecuenteque curse con paresia de pares craneales (PC) bajos y delVI PC. Sin embargo, la neuropatía del III PC es excepcional.Caso clínico. Hombre de 43 años que consulta por cefaleay visión doble de evolución subaguda y perfil ortostático.En la exploración destaca paresia del III PC izquierdosin afectación pupilar. En la resonancia magnética (RM) craneoespinalse observan hallazgos típicos que confirman lasospecha clínica de SHIC. El estudio del LCR muestra presiónde apertura de 5 cm de agua, leve proteinorraquia (0,63 g/l)y pleocitosis linfocitaria (13 leucocitos/ml). Un traumatismoraquídeo acontecido 3 meses antes de iniciarse la clínicacon demostración de fracturas vertebrales lumbares en tomografíacomputarizada de columna indica el probable origende la fuga de LCR. El cuadro se resolvió con tratamientoconservador en 3 meses. Una RM craneal realizada a los10 meses fue normal.Discusión. La neuropatía del III PC en el contexto delSHIC es una circunstancia excepcional que contribuye a ampliarsu espectro clínico (AU)

Introduction. Intracranial hypotension syndrome (IHS)is clinically defined by orthostatic headache due to cerebrospinalfluid (CSF) hypovolemia. It is frequently associatedwith lower cranial nerve (CN) paresis, especiallythe 6th one. However, neuropathy of the 3rd CN is rare.Case report. A 43 year old man presented with orthostaticheadache and vertical diplopia of gradual onset.Clinical examination detected incomplete, pupil sparingleft third CN palsy. Magnetic resonance imaging (MRI) ofthe brain and spine demonstrated typical abnormalitiesthat confirmed the clinical suspicion of IHS. Lumbarpuncture showed an opening pressure of 5 cm of water.CSF disclosed mild protein elevation (0.63 g/l) and lymphocyticpleocytosis (13 cells/ml). A history of spinaltrauma three months prior to the onset of the symptomsand lumbar fractures identified in a spine computedtomography scan suggested the likely origin of the CSFleak. The symptoms responded to conservative measuresin three months and a cranial MRI performed ten monthslater was normal.Discussion. Third CN palsy is an extremely unusualmanifestation of IHS and contributes to the expansion ofits clinical spectrum (AU)

[Third cranial nerve palsy due to intracranial hypotension syndrome]. / Neuropatía del tercer par craneal secundaria al síndrome de hipotensión intracraneal.

INTRODUCTION: Intracranial hypotension syndrome (IHS) is clinically defined by orthostatic headache due to cerebrospinal fluid (CSF) hypovolemia. It is frequently associated with lower cranial nerve (CN) paresis, especially the 6th one. However, neuropathy of the 3rd CN is rare. CASE REPORT: A 43 year old man presented with orthostatic headache and vertical diplopia of gradual onset. Clinical examination detected incomplete, pupil sparing left third CN palsy. Magnetic resonance imaging (MRI) of the brain and spine demonstrated typical abnormalities that confirmed the clinical suspicion of IHS. Lumbar puncture showed an opening pressure of 5 cm of water. CSF disclosed mild protein elevation (0.63 g/l) and lymphocytic pleocytosis (13 cells/ml). A history of spinal trauma three months prior to the onset of the symptoms and lumbar fractures identified in a spine computed tomography scan suggested the likely origin of the CSF leak. The symptoms responded to conservative measures in three months and a cranial MRI performed ten months later was normal. DISCUSSION: Third CN palsy is an extremely unusual manifestation of IHS and contributes to the expansion of its clinical spectrum.

Optociliary shunt vessels in compressive optic neuropathy by the intracranial internal carotid artery.

PURPOSE: To report a clinical case of optic nerve compression by supraclinoidal internal carotid artery associated with optociliary shunt vessels. METHODS: A 78-year-old woman with the clinical triad of left visual loss, ipsilateral optic disc pallor, and retinochoroidal (optociliary) shunt vessels is reported. She complained of loss of vision in the left eye of 2 years'' duration. RESULTS: A diffuse depression of the visual field was found in the affected eye. Magnetic resonance imaging revealed left optic nerve compression by the supraclinoidal internal carotid artery. CONCLUSIONS: The occurrence of optociliary shunt vessels, visual loss, and optic atrophy is a nonspecific sign of chronic optic nerve compression and in some instances may be falsely localized.

Perineural invasion through the maxillary division of the right trigeminal nerve in a rare case of nasolabial malignant peripheral nerve sheath tumor.

SUMMARY: We report the CT and MR imaging findings in a patient with malignant peripheral nerve sheath tumor in the right nasolabial area, which exhibited typical imaging features of cystic adenoid carcinoma.