Transcranial Sonography Characteristics of Cerebellar Neurodegenerative Ataxias.

Cerebellar neurodegenerative ataxias are a group of disorders affecting the cerebellum and its pathways with different neurological structures. Transcranial sonography (TCS) has been used for the evaluation of brain parenchymal structures in various diseases because of its fast and safe utilization, especially in neuropsychiatric and neurodegenerative diseases. The aim of our study was to investigate TCS characteristics of patients with neurodegenerative cerebellar ataxias. In our study, we included 74 patients with cerebellar degenerative ataxia; 36.5% had autosomal dominant onset, while 33.8% had sporadic onset. Standardized ultrasonographic planes were used for the identification of brain structures of interest. The SARA, INAS, neuropsychological and psychiatric scales were used for the further clinical evaluation of our study participants. The brainstem raphe was discontinued in 33.8% of the patients. The substantia nigra (SN) hyperechogenicity was identified in 79.7%. The third and fourth ventricle enlargement had 79.7% and 45.9% of patients, respectively. A positive and statistically significant correlation was found between SN hyperechogenicity with dystonia (p < 0.01), rigidity and dyskinesia (p < 0.05). The higher SARA total score is statistically significantly correlated with the larger diameter of the III (r = 0.373; p = 0.001) and IV ventricles (r = 0.324; p = 0.005). In such patients, the echogenicity of substantia nigra has been linked to extrapyramidal signs, and raphe discontinuity to depression. Furthermore, ataxia and its clinical subtypes have positively correlated with the IV ventricle diameter, indicating brain atrophy and brain mass reduction.

Non-Pharmacological Treatment of Primary Headaches-A Focused Review.

Headache disorders are a significant global health burden, leading to reduced quality of life. While vast pharmacological treatments are available, they may be associated with adverse effects or inadequate efficacy for some patients, therefore there is a need for exploring alternate treatment strategies. This review gives a brief explanation and evaluation of some established and emerging non-pharmacological approaches for headache management, focusing on nutraceuticals and diet, acupuncture, cognitive behavioral therapy (CBT), biofeedback, relaxation techniques, autogenic training, and neuromodulation. Special consideration is given to psychological interventions as they increase patient self-efficacy and provide strategies for managing chronic pain. Future research should focus on optimizing these therapies, identifying patient-specific factors influencing their effectiveness, and integrating them into holistic headache management strategies.

Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review.

Cerebral amyloid angiopathy-related inflammation (CAA-rI) is a largely reversible, subacute encephalopathy, which is considered as a rare variant of cerebral amyloid angiopathy (CAA). Although the diagnosis of this inflammatory vasculopathy is generally clinico-pathologic, a probable or possible diagnosis can often be established based on current clinico-radiological diagnostic criteria. This is important since CAA-rI is considered as a treatable disorder, which most commonly occurs in the elderly population. Behavioral changes and cognitive deterioration are highlighted as the most common clinical signs of CAA-rI, followed by a heterogeneous spectrum of typical and atypical clinical presentations. However, despite the well-established clinical and radiological features incorporated in the current diagnostic criteria for this CAA variant, this rare disorder is still insufficiently recognized and treated. Here, we have shown three patients diagnosed with probable CAA-rI, with significant heterogeneity in the clinical and neuroradiological presentations, followed by different disease courses and outcomes after the introduction of immunosuppressive treatment. Moreover, we have also summarized up-to-date literature data about this rare, yet underdiagnosed, immune-mediated vasculopathy.

Myasthenia gravis treated in the neurology intensive care unit: a 14-year single-centre experience.

INTRODUCTION: Severe myasthenia gravis (MG) exacerbation with respiratory failure and/or dysphagia usually requires monitoring and treatment in the neurology intensive care unit (NICU). The aim of our study was to identify all patients with severe MG exacerbation treated in the NICU in order to assessed potential factors affecting patients' need for mechanical ventilation, occurrence of complications and the final outcome. METHODS: We retrospectively included all patients with severe exacerbation of MG who required management in the NICU during a 14-year period. Baseline sociodemographic and clinical features, data on medication, comorbidities and outcome were obtained by reviewing medical records and institutional databases. RESULTS: Our study comprised 130 severe MG exacerbations detected in 118 patients. Median age of patients was 61.5 years, and women accounted for 58.5% of the patients. Half of the patients required mechanical ventilation during hospitalization. Lethal outcome was observed in 12.3% of severe MG exacerbations. Only elder age was an independent negative predictor of survival (OR 0.89, 95% CI 0.82-0.97, p < 0.01). Complications during hospitalization were detected in 50% of patients. A higher number of comorbidities (OR 1.09, 95% CI 1.60-2.35, p = 0.01) and mechanical ventilation (OR 28.48, 95% CI 8.56-94.81, p < 0.01) were independent predictors of complications during hospitalization. CONCLUSION: Patients with a severe MG exacerbation who do not require mechanical ventilation have a good outcome after treatment in the NICU. Elder age is an independent predictor of lethal outcome in patients with severe MG exacerbation. Mechanical ventilation and a higher number of comorbidities lead to more frequent complications.

Non-convulsive status epilepticus as an initial manifestation of herpes simplex virus encephalitis.

Herpes simplex encephalitis (HSVE), manifesting with non-convulsive status epilepticus (NCSE), normocellular CSF findings and CT features of acute ischemic stroke, is a rare finding that can be hard to diagnose accurately. We present a case of HSVE and compare our results to those of previously published cases with the same pathology, in order to provide information to support more rapid and effective diagnosis and treatment. A Pubmed search of reported cases was conducted and five cases of HSVE manifesting with NCSE were found. Each of the cases, including ours, was compared in terms of clinical manifestations and CSF, CT and EEG findings. The clinical manifestations in our patient correlated with those of the other cases. EEG showing sharp fronto-temporo-centro-parietal waves was only observed in our patient. Similar CT manifestations were found in one other patient, normocellular CSF was registered in three other cases and positive PCR for HSV in four patients. Information about the possible clinical manifestations and CT, EEG and CSF findings in patients with HSVE manifesting with NCSE is crucial for a fast diagnosis and successful treatment, leading to higher survival rates and fewer complications and neurological deficits.