Clin. Transl. Neurosci., Volume 3, Issue 2 (September 2019) – 10 articles

The aetiology of phrenic neuropathy is often unknown, but immune mechanisms may play a role. In a typical case of bilateral phrenic neuropathy with paradoxical breathing (video), an inflammatory pathogenesis was suggested by prolonged distal latency of phrenic nerve compound muscle action potentials in nerve conduction studies and a clear-cut albumin-cytologic dissociation. This encouraged us to treat the patient with a standard dose of intravenous immunoglobulin. After obtaining a strong improvement at spirometry, we repeated the second cycle of intravenous immunoglobulin and observed normalization of symptoms within few weeks and no relapse after 3 years. This case suggests that lumbar puncture should be performed in the acute phase of phrenic neuropathies to detect potential responders to immunomodulatory treatment. Full article

Objective: Meningiomas represent the most common intracranial extraaxial neoplasia in adults, accounting for a third of all diagnosed primary tumors of the brain. Despite decades of research, relatively little data on demographics of meningiomas exist. The aim of our study was to undertake an analysis of demographics and outcome of patients who underwent meningioma surgery over an 8-year time period at our institution. Methods: We reviewed 187 consecutive patients with histologically confirmed meningioma. Demographic data, tumor location and side, surgical resection grade, and histopathological and radiological data were collected and assessed for all patients. Furthermore, recurrence, morbidity, and mortality were evaluated. Results: Of the 187 consecutive patients undergoing meningioma resection over a period of 8 years, 131 (70.1%) were women (p < 0.001). Meningiomas were classified as World Health Organization (WHO) grade I, grade II, and grade III in 66.8%, 31.0%, and 2.1%, of the cases, respectively (p < 0.001). MIB-1 proliferation index was <1 in 7.5%, 1–5 in 52.9%, 6–10 in 22.4%, >10 in 11.8% of the lesions (p < 0.001). In 82.4% of the cases, gross total resection was achieved. Recurrence occurred in 23 patients (12.3%), while overall morbidity and mortality rate was 41.2% and 7.7%, respectively. Conclusion: Based on our results, women are more than twice as likely to be affected, and the peak age is between 60 years and 70 years. Recurrence rate in our cohort was relatively low when compared to the data in the literature. The diagnosis of WHO grade II meningiomas, 31% in our cohort, is increasing since the 2007 WHO criteria have been published. Full article

In the initial assessment of the patient with acute vertigo or dizziness, both structured history-taking and a targeted bedside neuro-otological examination are essential for distinguishing potentially life-threatening central vestibular causes from those of benign, self-limited peripheral labyrinthine origin and thus for deciding on further diagnostic testing. In this article, the key elements of the vestibular and ocular motor examination, which should be obtained at the bedside in these acutely dizzy patients, will be discussed. Specifically, this will include the following five domains: ocular stability for (I) nystagmus and for (II) eye position (skew deviation), (III) the head-impulse test (HIT), (IV) postural stability, and (V) ocular motor deficits of saccades, smooth pursuit eye movements, and optokinetic nystagmus. We will also discuss the diagnostic accuracy of specific combinations of these bedside tests (i.e., HIT, testing for nystagmus and vertical divergence, referred to as the H.I.N.T.S. three-step examination), emphasizing that the targeted neuro-otological bedside examination is more sensitive for identifying central causes in acute prolonged vertigo and dizziness than early MRI of the brain. Full article

In great part due to recent advances in medical technology, ischemic cerebral stroke has become an increasingly treatable condition, which requires urgent measures and which rely on pharmacological and/or interventional measures. Due to its high prevalence, preventive measures should also be undertaken, and this is a situation where the use of advanced neuroimaging techniques could be helpful in certain underlying diseases. What is proposed here is to discuss how modern neuroimaging techniques (advanced magnetic resonance (MR) techniques and/or nuclear medicine techniques such as positron emission tomography (PET)) could help in situations that would otherwise lead to a stroke. Since both primary and secondary prevention measures are often required, we see that the techniques can be helpful in both situations. The diseases that cause stroke that can be investigated are, among others, carotid stenosis; transient ischemic attacks (TIAs) may also be followed by a major stroke if nothing is undertaken. It has been established that carotid stenosis is to be treated in the presence of a significant lesion that has neurological symptoms. The question of how to deal with these patients often arises when the relationship between the stenosis and symptoms is not significant or the symptomatology unclear. In such situations, either PET and/or fat-saturated T1 images of the carotids can help to demonstrate the embolic nature of the plaque. We have seen that carotid plaque vulnerability, which can cause embolism, can be associated with plaque inflammation (seen on PET) or plaque haemorrhage (seen on MR images). Also, while PET and MRI will demonstrate different stages of plaque vulnerability, they can both help to demonstrate vascular lesions that are at risk of causing significant ischemic events. Diffusion-weighted imaging (DWI) has shown that some TIAs may indeed be ischemic brain lesions with a transient symptomatology. The early use of DWI can thus help treat these patients more acutely. Based on this, we have seen that newer imaging techniques can provide additional knowledge about conditions that may lead to stroke and be treated. This should have a major impact on patient outcomes and ultimately on healthcare costs related to this condition. Full article

Fulgence Raymond (1844–1910) succeeded Jean-Martin Charcot (1825–1893) to the Chair of Nervous System Diseases. As famous as Charcot remains, Raymond has been forgotten. After a brief biographical account, we will present a few examples of his work still relevant today: hemichorea, Raymond-Cestan syndrome, hereditary spastic paraplegia and acute ascendant paralysis. In each case, his accurate clinical and anatomopathological descriptions are accompanied by aetiological hypotheses that are remarkably prescient with regard to current knowledge. Strongly committed to teaching, he published most of his lessons every year. They remain highly relevant historically, and sometimes for other reasons, as we shall see. We hope to show that Raymond does not deserve to be forgotten. Full article

Neurorehabilitation is a rapidly developing subspecialty of neurology due to medical advances and growing knowledge on functional recovery from brain injury such as plasticity and regeneration in the nervous system. Furthermore, progress in modern technologies facilitate new therapeutic concepts. Patient-tailored, flexible multimodal neurorehabilitation is essential in neurological diseases due to the diversity of symptoms. In addition, rehabilitative treatment should be realized from disease onset. To fulfill these goals, the neurocenter of the Cantonal Hospital Lucerne established an uninterrupted treatment chain from the emergency stage to the social and occupational reintegration, which is described in this article with a focus on stroke, Parkinson’s disease, and multiple sclerosis patients. Full article

We report the case of a 58-year-old Caucasian woman who presented with a subacute cerebellar syndrome accompanied by disturbance of the hypothalamic–pituitary axis and was diagnosed with isolated neurosarcoidosis based on radiological findings including typically located cerebral lesions (infratentorial and pituitary stalk). Due to persistent clinical and radiological disease activity during several years despite escalation of immunosuppressive treatment, the diagnosis was reevaluated, and a transsphenoidal biopsy of a lesion at the pituitary stalk was performed revealing Langerhans cell histiocytosis. In this case, we discuss the different steps leading to the diagnostic error, as well as the presence of red flags, which should have led to an earlier diagnostic reevaluation. Full article

Positron emission tomography (PET) has a long history in the assessment of brain physiology and pathology; however, its initial use was limited to more academic and scientific settings. This changed radically with the clinical introduction of PET–computed tomography (PET-CT). PET-CT has become an integral part of the radiological armamentarium, especially in the assessment of patients with oncological or cardiovascular disorders. It has until now not found a clear role in neuroradiology. Indeed, magnetic resonance techniques have proven superior for the investigation of brain tumors and inflammatory diseases. However, some newer applications are emerging that can re-center PET-CT clearly in the neuroradiological world such as the investigation of vascular diseases. Full article