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.

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.

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.

[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).

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.

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.

Laryngeal tuberculosis in renal allograft patients.

Laryngeal tuberculosis, although the most common granulomatous disease of the larynx, is a rare form of extrapulmonary tuberculosis, never reported in immunosuppressed allograft recipients. We present two cases of laryngeal tuberculosis in renal transplant patients and a review of the literature. Two women, a 29-year-old and a 60-year-old, each more than 9 years after their cadaveric renal allograft, presented with a 2-week febrile illness with hoarseness and dysphagia, and both were found to have laryngeal tuberculosis by direct laryngoscopy. Although both radiographs were unremarkable, both patients had sputum positive for acid-fast bacilli that subsequently grew Mycobacterium tuberculosis. Clinical response promptly followed institution of isoniazid, rifampicin, and pyrazinamide in each case, although both required threefold increases in daily cyclosporin A dosage to maintain therapeutic levels.

Intramedullary cysticercosis cured with drug treatment. A case report.

STUDY DESIGN: A report of a patient with cervical intramedullary cysticercosis is presented. OBJECTIVES: To report the first case of intramedullary. cysticercosis cured with drug management only, which supports the hypothesis that management with anthelmintics might be successfully used in this form of cysticercosis. SUMMARY OF BACKGROUND DATA: Intramedullary cysticercosis is a rare manifestation of neurocysticercosis. Every treated patient reported to date has undergone surgery, frequently necessary for diagnosis. Since the anthelmintics praziquantel and albendazole were shown to be effective in parenchymal brain cysticercosis, these drugs have been considered potentially useful in patients with intramedullary cysticercosis. Nevertheless, no case yet had been reported to be cared with only medical therapy. METHODS: The treatment of a patient who suffered multiple cysticercal reinfestations of the nervous system is presented. RESULTS: The patient received prolonged treatment with albendazole because of superimposed cerebral reinfestations. During this treatment, she suffered acute paraparesis, and cervical magnetic resonance imaging showed cyst-like lesions with linear gadolinium enhancement and perilesional edema, indicative of dying cysticerci and inflammatory host reaction. Dexamethasone was added, and progressive neurologic improvement followed with complete resolution of intramedullary lesions. CONCLUSIONS: A preoperative diagnostic suspicion of cysticercosis is important in patients with intramedullary cystic lesions because specific drug treatment is available. Treatment with anthelmintics, particularly albendazole, should be considered in patients with intramedullary cysticercosis before surgery. Corticosteroids may be added to the therapeutic regimen because this may reduce the perilesional edema and prevent neurologic deterioration during the course of anthelmintic treatment.

Parapharyngeal emphysema--an unusual complication of temporal bone trauma.

We report a case of a 36-year-old woman who suffered a temporal bone trauma due to a bicycle crash. Computed tomography revealed parapharyngeal emphysema despite only minor temporal bone changes.

[Spinal cord compression secondary to epidural extramedullary hematopoiesis in thalassemia: a clinical case and review of literature]. / Compresión medular secundaria a hematopoyesis extramedular epidural en talasemia: caso clínico y revisión de la literatura.

INTRODUCTION: Extramedullary hematopoiesis generally occurs in a variety of hematological disorders where the normal functioning of the blood forming organs is disturbed. It is a common manifestation in thalassemia where it occurs as a compensatory phenomenon in order to combat long standing anemia. Spinal cord compression as a consequence of extramedullary hematopoiesis in the intraspinal epidural space is an extremely rare complication, though this complication has been reported more commonly in thalassemia. CLINICAL CASE: A case of spinal cord compression due to extramedullary hematopoiesis in a patient with thalassemia is reported. The patient was successfully treated with radiation therapy and blood transfusions and he made a complete clinical recovery. Development. The literature is reviewed and the efficacy of several treatments such as surgery, radiotherapy and blood transfusion therapy is discussed. Until recently surgical decompression followed by radiation therapy remained the recommended treatment. Hematopoietic tissue is particularly sensitive to the ionizing radiation and low-dose radiotherapy is enough to relieve the spinal cord compression. Blood transfusion therapy may be diagnostically and therapeutically useful in the management of this entity. CONCLUSIONS: A prompt recognition of the syndrome and early treatment with radiotherapy and blood transfusion therapy is recommended to prevent irreversible damage to the spinal cord. Surgery may be only considered in the event of progressive neurological deficit despite of radiotherapy or blood transfusion therapy.

[Progressive amyotrophy of a limb as the presenting symptom of anchored spinal cord syndrome with spinal lipoma]. / Amiotrofia progresiva de una extremidad como síntoma de presentación del síndrome de médula anclada con lipoma espina.

OBJECTIVE: We present a case of progressive amyotrophy of a limb as the presenting symptom of the anchored spinal cord syndrome and review the principal clinical features of the syndrome, diagnostic tests which are useful in differentiating it from other conditions and its treatment. CLINICAL CASE: We describe a case of a young woman in whom the spinal cord was anchored by a spinal lipoma. At the onset of the disorder she complained of progressive muscular atrophy of the right leg and difficulty in dorsi-flexion of her right foot. Plain X-ray of the pelvis showed partial agenesis of the right lower hemisacrum and partial sacralization of L5. Lumbosacral CT and MR showed a lipoma to be present within the spinal canal and the thickened filum terminale anchored within the lipoma. During the next five months after diagnosis, the clinical picture worsened with paresia of flexo-extension of the right knee and of flexion of the right foot. Surgical treatment was therefore indicated. CONCLUSIONS: The anchored spinal cord syndrome should be considered in the differential diagnosis of spinal cord disorders presenting in adults, when there are other malformations such as agenesis of the sacrum. Surgical treatment is always indicated when there is evidence of worsening clinical condition.

[Diagnostic evaluation of nasopharyngeal carcinoma: role of Epstein-Barr virus]. / Evaluación diagnóstica del carcinoma nasofaríngeo: papel del virus de Epstein-Barr.

We present a retrospective series of 27 nasopharyngeal carcinomas, selected from those attended at Ramón y Cajal Hospital between 1977 and 1996, with the aim of review the role of the study of Epstein-Barr virus in the diagnostic process of nasopharyngeal carcinoma. Twenty-seven patients, ranging from 14 to 81 years, with an average age of 50 years were selected. Male/female ratio was 1,7. All but one case were Caucasian. A neck mass was the first symptom in 40% of cases, with a mean diagnostic delay of 17 months. Only 8 cases (23%) did not exhibit neck nodes at the moment of diagnosis. CT and MRI were essential to establish staging: 5 stage I, 7 stage II and 15 stage IV, due to regional extension and/or bone erosion. Radiotherapy was employed in all cases, helped by chemotherapy in 20% of them. With a mean follow-up of 62 months, 5-years survival was 32% (IC 14,06-52,09). Of 27 cases of nasopharyngeal carcinoma 4 were differentiated (type I), 2 moderately differentiated (type II) and 22 undifferentiated (type III). While LMP-1 was only expressed by 41% of cases, PCR detected Epstein-Barr virus genome in 26 cases (96%) and in situ hybridization for EBERs was positive in all cases. Thus, all nasopharyngeal carcinomas were related to Epstein-Barr virus. Expression of LMP-1 seemed to worse the prognosis of nasopharyngeal carcinoma.

[Temporal bone fracture: review of 40 cases (44 traumatic injuries)]. / Fracturas del hueso temporal. Revisión de 40 casos (44 traumatismos).

Temporal bone fractures are common injuries in patients suffering cranio-encephalic traumatisms. They call for a precociuos diagnosis, including a careful oto-neurologic examination as well a computerized tomography (CT), in order to asses sequelae and complications arrised from traumata. Here are reported a review of 40 patients with 44 temporal fractures attended in our Hospital, from January 1993 until March 1996. The injured were clinically evaluated in the ENT-Department by computer radiography and the classic audiovestibular battery tests. Particularly emphasized were luxations of the ossicular chain, lesion of the labyrinthine capsula and peripheral facial palsies. Our basic concern is to evaluate the incidence of petrosal involvement in our environment, owing to the scarcity of the national literature, and to analyze the after maths and the management of postraumatic facial paralyses.

[Maxillary mucocele: two case reports and review of literature]. / Mucocele maxilar. Presentación de dos casos y revisión de la literatura.

Mucoceles are benign expansive conditions, chronically evolving, from paranasal sinuses. Most frequent localized in the ethmofrontal area and related with sinusal ostial drainage. Maxillary mucoceles are rarer and related with traumatic, tumoral or surgical pathologies, particularly with the Caldwell-Luc incision. These mucoceles demand an aggressive treatment als those etmofrontals in order to achieve a radical exeresis through an outer incision. We report 2 cases of maxillary mucocele. The first was a big one of idiopathic type, the other was associated to an inverted papilloma sitting in the external wall of the affected nasal cavity. Review of the literature in regard of pathogenesis, radiological diagnosis and management of these cases.

[Pott's puffy tumor]. / Tumor blando de Pott.

Between the complications of frontal sinusitis orbital or intracranial are the most frequent encountered (meningitis, abscesses and empyemas). All are secondary to thrombophlebitis of veins communicating the intracranial cavity with the frontal sinus. Frontal osteomyelitis secondary to sinusitis, the so-called Pott's puffy tumor, is a much more rare aftermath in the antibiotic epoch. Pott's puffy tumor must be suspected in patients with frontal headache followed by frontal oedema. Concerning the diagnosis clinical suspicion is essential and must be settled throughout computerized tomography and/or magnetic resonance or even bone scintiscan. The paper report 2 cases, one an orbital periostitis, at the beginning of the disease, which was recovered with medical antibiotic treatment and another one, an osteomyelitis somewhat evolved requiring surgery through frontal osteoplasty. Perusal of etiology, pathogenesis, diagnosis and treatment of this complication.

[Nasal encephalocele: differential diagnosis with nasal glioma]. / Encefalocele nasal: diagnóstico diferencial con el glioma nasal.

Congenital nose neoplasms are infrequent, between them neurogenic tumors of the middle line include nasal gliomata (glial ectopies) and nasal encephaloceles, according to an existing or lacking communicating link with the intracranial cavity. We report one congenital naso-encephalocele case in a 16-year-old girl suffering from repeated meningitis events after several nasal polypectomies performed in other departments. She underwent complete removal through fronto-neurosurgical and paralateronasal approach, being the young woman asymptomatic for 3 years. We discuss about both intranasal gliomata and encephaloceles, certainly of not easy pathologic identification, stressing the decisive value of imaging techniques, as magnetic resonance, in order to clearcut both the diagnosis and the therapeutical planning.