Neurological Research and Practice最新文献

Introduction: Chronic headache due to the overuse of medication for the treatment of migraine attacks has a prevalence of 0.5-2.0%. This guideline provides guidance for the management of medication overuse (MO) and medication overuse headache (MOH).

Recommendations: Treatment of headache due to overuse of analgesics or specific migraine medications involves several stages. Patients with medication overuse (MO) or medication overuse headache (MOH) should be educated about the relationship between frequent use of symptomatic headache medication and the transition from episodic to chronic migraine (chronification), with the aim of reducing and limiting the use of acute medication. In a second step, migraine prophylaxis should be initiated in patients with migraine and overuse of analgesics or specific migraine drugs. Topiramate, onabotulinumtoxinA and the monoclonal antibodies against CGRP or the CGRP-receptor are effective in patients with chronic migraine and medication overuse. In patients with tension-type headache, prophylaxis is performed with amitriptyline. Drug prophylaxis should be supplemented by non-drug interventions. For patients in whom education and prophylactic medication are not effective, pausing acute medication is recommended. This treatment can be performed in an outpatient, day hospital or inpatient setting. Patients with headache due to overuse of opioids should undergo inpatient withdrawal. The success rate of the stepped treatment approach is 50-70% after 6 to 12 months. A high relapse rate is observed in patients with opioid overuse. Tricyclic antidepressants, neuroleptics (antiemetics) and the administration of steroids are recommended for the treatment of withdrawal symptoms or headaches during the medication pause. Consistent patient education and further close monitoring reduce the risk of relapse.

Introduction: Super-refractory status epilepticus (SRSE) represents the culmination of refractory status epilepticus (RSE) and carries a significant risk of poor neurological outcome and high mortality. RSE is not defined primarily by seizure duration, but by failure to respond to appropriate antiseizure treatment. SRSE is present when a RSE persists or recurs after more than 24 h of treatment with anesthetics. No evidence-based treatment algorithms can be provided for SRSE. Therefore, we propose a pragmatic standard operating procedure (SOP) for the management of SRSE that addresses the existing uncertainties in the treatment of SRSE and provides options for resolution and decision-making.

Comments: First, we recommend the assessment of persistent seizure activity and the evaluation of differential diagnoses to confirm correct diagnosis. Relevant differential diagnoses include psychogenic non-epileptic seizures, hypoxic, metabolic, or toxic encephalopathies, and tetanus. During SE or in severe encephalopathies, a so-called electroclinical ictal-interictal continuum may occur, which denotes an intermediate stage that cannot be defined with certainty as ictal or interictal by EEG and should not lead to harmful overtreatment. Because both prognosis and specific treatment options depend crucially on the etiology of SRSE, the etiological evaluation should be performed rapidly. When SRSE is confirmed, various pharmacological and non-pharmacological treatment options are available.

Conclusion: We provide a pragmatical SOP for adult people with SRSE.

Background: Hypoglycemia in patients with diabetes mellitus, particularly type 1 can mimic acute ischemic stroke by causing focal neurological deficits. In acute ischemic stroke, the interpretation of emergency imaging including computed tomography with angiography and perfusion is crucial to guide revascularizing therapy including intravenous thrombolysis. However, different metabolic abnormalities and stroke mimics can cause focal hypoperfusion.

Methods: We describe two type 1 diabetes patients presenting with acute focal neurological deficits and hypoglycemia, who underwent multimodal computed tomography and follow-up imaging.

Case presentation: Patient 1, a 20-year-old man presented with aphasia and interstitial glucose level of 54 mg/dl. Patient 2, a 77-year-old man presented with aphasia, mild right-sided brachiofacial paresis and interstitial glucose level of 83 mg/dl. On brain imaging, no acute infarct signs were noted. Yet, both had focal left hemispheric cerebral hypoperfusion without large-vessel occlusion or stenosis. Due to persistent symptoms after normalization of blood glucose and despite a perfusion imaging pattern that was interpretated as non-typical for ischemia, both patients underwent thrombolysis without any complications.

Conclusion: Computed tomography perfusion might help to discriminate hypoglycemia with focal neurological signs from acute stroke, but further evidence is needed.

Background: No controlled studies for non-systemic vasculitic neuropathy treatment exist (NSVN). We compared the treatment response to induction therapy commonly used in clinical practice in NSVN.

Methods: In this retrospective single-center study, 43 patients with biopsy-proven NSVN were analyzed. Patients were subdivided into groups depending on their initial treatment. Relapse rates, changes of motor and sensory symptoms, adverse events, predictors of relapses, and second-line treatment were compared.

Results: Initial treatment regimens were corticosteroid monotherapy, cyclophosphamide monotherapy, pulsed corticosteroid therapy, and combination therapy. Discontinuation due to adverse events occurred in 6 of 43 patients. Clinical data did not differ between treatment groups. Within 12 months, 24.3% of patients relapsed. The median time to relapse was 4 (1.5, 6) months. No relapse occurred in the combination therapy group. However, there was no statistically significant difference in relapse-free survival between treatment groups (p = 0.58). Neither clinical data nor biopsy analysis predicted relapses sufficiently. As a second-line treatment, cyclophosphamide as mono- or combination therapy was used (7 of 9 patients) most frequently. One patient was treated with methotrexate, and one with IVIG.

Conclusions: Induction therapy used in clinical practice is effective and mainly well-tolerated in NSVN. Our data do not support an overall advantage of cyclophosphamide over corticosteroid monotherapy. Controlled trials comparing the effectiveness of induction and maintenance therapy in NSVN are warranted.

Adenylyl cyclase 5 (ADCY5) related dyskinesia is a rare disorder characterized by early-onset paroxysmal choreoathetosis, dystonia, myoclonus, or a combination of the above, which primarily involved the limbs, face, and neck. Other common clinical features are axial hypotonia and episodic exacerbation of dyskinesia. Both sporadic and inherited cases have been reported and the predomiant mode of inheritance is autosomal dominant. Herein, we describe the first ADCY5-related dyskinesia patient in Taiwan.

In order to inspire and attract young people to Neurology, we must offer high-quality and attractive teaching! To improve neurological education at our Medical School (Technical University of Munich), we converted the main lecture into an e-learning concept using a flipped classroom model. Students had to prepare with a video and a text as well as answering multiple choice questions before each lecture. As a further incentive, students with ≥ 80% right answers in multiple choice questions received a bonus for the final exam. During the lectures, predominantely patient cases were discussed to apply, improve and enhance the previously acquired knowledge. The realignment of the main lecture in Neurology into a flipped classroom model was very successful and was further optimized in the following semesters based on the evaluations obtained for the new concept. Moreover, this enabled us to quickly switch to remote teaching during the COVID-19 pandemic, while still offering lectures of high quality. In addition, this new teaching concept attracts students for Neurology. Furthermore, the exemplary conversion of the Neurology main lecture to a flipped classroom concept also serves as best practice and motivation to adapt other courses in our faculty and far beyond.

Background: Central retinal artery occlusion (CRAO) is a neuro-ophthalmological emergency whose optimal management is still under debate and due to the absence of definite guidelines, practice is expected to vary. We aimed to characterize early evaluation as well as acute treatment and diagnostic approaches in German hospitals with a stroke unit (SU).

Methods: In 07/2021, all 335 certified German SUs were invited to participate in an anonymous online survey endorsed by the German Stroke Society on emergency department care organization, diagnostic procedures, and treatment of patients with unilateral vision loss (UVL) subsequently diagnosed with CRAO.

Results: One hundred and sixty-three (48.6%) of the 335 eligible centers responded. Most (117/135; 86.7%) stated that UVL patients were treated as an emergency, in 62/138 (44.9%) hospitals according to specific guidelines. First-line evaluation was performed by neurologists in 85/136 (62.5%) hospitals, by ophthalmologists in 43/136 (31.6%) hospitals. Seventy of 135 (51.9%) respondents indicated a lack of on-site ophthalmological expertise. Seventy-four of 129 (57.4%) respondents performed thrombolysis in CRAO and 92/97 (94.8%) stated that patients with CRAO-if admitted to neurology-were treated on a SU.

Conclusions: Our findings reflect notable heterogeneity in early intrahospital care of CRAO in German SUs but demonstrate a preference for work-up and management as acute stroke by the involved neurologists. Streamlining interdisciplinary emergency evaluation is essential for ongoing and future prospective trials.

Levamisole is a common adulterant of cocaine and has been associated with reversible leukoencephalopathy in cocaine users. We report a case of two episodes with severe neurological symptoms and multifocal white matter lesions with brainstem and cerebellar involvement in a 29-year-old man after sporadic cocaine consumption. A urinalysis was positive for levamisole. Neurological deficits as well as MRI presentation improved after cessation of levamisole exposure and two courses of intravenous high-dose glucocorticoid therapy. Early diagnosis of levamisole-induced multifocal leukoencephalopathy and treatment with corticosteroids without delay is essential for a good recovery from neurological symptoms. Although cocaine is one of the most prevalent abused illicit drugs, cocaine- and levamisole-induced multifocal leukoencephalopathy is underdiagnosed as this disorder is not often described in the literature and anamnesis of drug abuse is not admitted by the patient. Therefore, an additional screening for cocaine and levamisole in clinical practice is useful in similar cases to support the diagnosis.

Introduction: Subacute cerebellar ataxia combined with cerebrospinal fluid (CSF) pleocytosis is the result of an immune response that can occur due to viral infections, paraneoplastic diseases or autoimmune-mediated mechanisms. In the following we present the first description of a patient with anti-Homer-3 antibodies in serum and CSF who has been diagnosed with paraneoplastic subacute cerebellar degeneration due to a papillary adenocarcinoma of the breast.

Case presentation: A 58-year-old female was admitted to our clinical department because of increasing gait and visual disturbances starting nine months ago. The neurological examination revealed a downbeat nystagmus, oscillopsia, a severe standing and gait ataxia and a slight dysarthria. Cranial MRI showed no pathological findings. Examination of CSF showed a lymphocytic pleocytosis of 11 cells/µl and an intrathecal IgG synthesis of 26%. Initially, standard serological testing in serum and CSF did not indicate any autoimmune or paraneoplastic aetiology. However, an antigen-specific indirect immunofluorescence test (IIFT) revealed the presence of anti-Homer-3 antibodies (IgG) with a serum titer of 1: 32,000 and a titer of 1: 100 in CSF. Subsequent histological examination of a right axillary lymph node mass showed papillary adenocarcinoma cells. Breast MRI detected multiple bilateral lesions as a diffuse tumour manifestation indicative of adenocarcinoma of the breast. Treatment with high-dose methylprednisolone followed by five plasmaphereses and treatment with 4-aminopyridine resulted in a moderate decrease of the downbeat nystagmus and she was able to move independently with a wheeled walker after 3 weeks. The patient was subsequently treated with chemotherapy (epirubicin, cyclophosphamide) and two series of immunoglobulins (5 × 30 g each). This resulted in a moderate improvement of the cerebellar symptoms with a decrease of ataxia and disappearance of the downbeat nystagmus.

Conclusion: The presented case of anti-Homer-3 antibody-associated cerebellar degeneration is the first that is clearly associated with the detection of a tumour. Interestingly, the Homer-3 protein interaction partner metabotropic glutamate receptor subtype 1A (mGluR1A) is predominantly expressed in Purkinje cells where its function is essential for motor coordination and motor learning. Based on our findings, in subacute cerebellar degeneration, we recommend considering serological testing for anti-Homer-3 antibodies in serum and cerebrospinal fluid together with tumor screening.

Background: The ongoing expansion of the cosmetic armamentarium of facial rejuvenation fails to uncover the inherent risks of cosmetic interventions. Informed consent to all risks of cosmetic filler injections and potential sequelae, including ocular and neurological complications, should be carefully ensured. We present two cases of complications following facial hyaluronic acid filler injections.

Case presentations: Case 1: A 43-year-old woman presented with monocular vision loss of the left eye, associated ptosis, ophthalmoplegia, periocular pain and nausea, cutaneous changes of the glabella region and forehead, and sensory impairment in the left maxillary branch dermatome (V2) after receiving a hyaluronic acid (HA) filler injection into the left glabellar area. On ophthalmological examination, an ophthalmic artery occlusion (OAO) was diagnosed upon identification of a "cherry-red spot". Magnetic resonance imaging (MRI) revealed a left ischemic optic neuropathy. Supportive therapy and hyaluronidase injections were initiated. A follow-up MRI of the head performed two months after presentation corresponded to stable MRI findings. The patient had irreversible and complete vision loss of the left eye, however, the ptosis resolved. Case 2: A 29-year-old woman was admitted to hospital a few hours after a rhinoplasty and cheek augmentation with hyaluronic acid, presenting with acute monocular vision loss in the right eye, retrobulbar pain, fatigue and vomiting. In addition, the patient presented a harbinger of impending skin necrosis and a complete oculomotor nerve palsy on the right side, choroidal ischemia and vision impairment. Supportive treatment and hyaluronidase injections into the ischemic tissue were initiated. A small scar at the tip of the nose, vision impairment and an irregular pupillary margin on the right side persisted at follow-up.

Conclusion: These two case reports and the literature review emphasize the pathophysiological mechanisms leading to potentially devastating complications. In order to reduce the risk of vision loss secondary to cosmetic filler injections, practitioners should possess a thorough knowledge of anatomy and preventive strategies.