eNeurologicalSci最新文献

Background

The incidence of Amyotrophic Lateral Sclerosis (ALS) varies among different geographical areas and seems to increase over time. This study aimed to examine the epidemiologic data of ALS in the north-east Tuscany and compare the results with those of similar surveys.

Methods

Data from ALS cases diagnosed in Florence and Prato Hospitals were prospectively collected from 1st June 2018 to 31st May 2021.

Results

The age- and sex-adjusted incidence rate of ALS in cases per 100,000 population was 2.71 (M/F ratio: 1.21), significantly higher as compared to that reported in the 1967–1976 decade in the same geographical area (0.714). The age- and sex-adjusted incidence rate among resident strangers was similar to that of the general population (2.69). A slightly higher incidence rate (4.36) was observed in the north-east area of Florence province, which includes the Mugello valley. The mean prevalence was of 7.17/100,00. The mean age at diagnosis was 69.7 years, with a peak between 70 and 79 years among men and a smoother age curve among women.

Conclusions

ALS epidemiological features in north-east Tuscany are in line with other Italian and European Centers. The dramatic increase of the local disease burden over the last decades probably reflects better ascertainment methods and health system.

The most common infection preceding Guillain–Barré syndrome (GBS) is Campylobacter jejuni enteritis, although a few patients present with Campylobacter coli. We report a case of C. coli–induced fulminant GBS. A 61-year-old woman presented with bilateral limb weakness. Nerve conduction studies revealed a reduction of amplitude and C. coli was isolated from a fecal specimen, leading to the diagnosis of GBS. Although the patient was immediately administered immunoglobulin, her symptoms rapidly worsened and she died. Peripheral nerve autopsy revealed myelin ovoid, and infiltration of CD68-positive macrophages into nerves. More effective treatments for fulminant GBS need to be developed.

Fibrodysplasia ossificans progressiva (FOP) is a rare, disabling genetic condition characterized by congenital malformations of the great toes and progressive heterotopic ossification. Here, we briefly describe the case of a 56-year-old male with known FOP and acute ischemic stroke that underwent mechanical thrombectomy with conscious sedation. Treating physicians should be aware of special medical considerations to prevent flare-ups and inflammation that result from any tissue injuries in this disease. Mechanical thrombectomy is a challenging scenario because general anesthesia and injections should be avoided in these patients. The treatment is still preventive and supportive, but this is the first report of the procedure in a patient with FOP.

Background

Spinal cord injury results in the interruption of neuronal conduction in the spinal cord, a condition that occurs in 0.1% of the world's population. This results in severe limitations in autonomy including locomotor function. Its recovery can be pursued through conventional isolated physiotherapeutic rehabilitation (overground walking training - OGT) or associated with Robot-assisted gait training - RAGT (e.g.: Lokomat ®).

Aim

The aim of this review is to compare the effectiveness of RAGT combined with conventional physiotherapy.

Methods

The databases consulted, from March 2022 to November 2022, were PubMed, PEDro, Cochrane Central Register of Controlled Trials (Cochrane Library) and CINAHL. RCT studies of people with incomplete spinal cord injuries treated with RAGT and/or OGT with the aim of improving walking were analysed.

Results

Among the 84 RCTs identified, 4 were included in the synthesis, with a total of 258 participants. The outcomes analysed concerned both locomotor function through lower limb muscle strength and the need for assistance in walking, using the WISCI-II scale and the LEMS. Robotic treatment stimulated the greatest improvements in the four studies; however, they were not always statistically significant.

Conclusion

A rehabilitation protocol combining RAGT with conventional physiotherapy is more effective than isolated OGT in improving ambulation in the subacute phase.

Background and purpose

Watershed infarcts denote ischemic lesions involving the distal territories of two major arteries. For years, hypotheses on its pathophysiological mechanisms have been proposed. Yet, the cause is still widely debated. This study aimed to determine the mechanism of watershed strokes and compare their clinical outcomes to acute ischemic stroke from other causes and predict the factors affecting clinical outcomes in patients with watershed infarcts.

Methods

This single-center, comparative, six-years retrospective cohort study included patients with a diagnosis of Acute Ischemic Stroke. Patients were classified under watershed group or acute ischemic stroke based on their neuroimaging findings. Stroke mechanisms were determined between groups as well as the factors associated with clinical outcomes in watershed strokes.

Results

Among the 424 patients included in the study, large artery atherosclerosis was seen in greater frequency in patients with watershed infarcts regardless of the type (EWIs: n = 68, 73% vs IWIs: n = 89, 75%). No differences observed in the clinical outcomes between groups. Multiple variable analysis showed that age, female sex, high NIHSS score and presence of underlying malignancy were associated with clinical outcomes.

Conclusion

Clinical outcomes between watershed infarcts and acute ischemic strokes were similar. Hemodynamic compromise in the setting of severe stenosis is the underlying mechanism for both types of watershed strokes thus, the goal of treatment is to maintain adequate perfusion. High baseline NIHSS score, increased age, female gender and underlying malignancy were all poor predictors of clinical outcomes in patients with watershed strokes.

A 78-year-old man developed paresthesias in the extremities. He was referred to our hospital because of positive anti-human T-cell leukemia virus type 1 (HTLV-1) antibodies in the serum and the presence of abnormal lymphocytes. He was diagnosed as chronic-type adult T-cell leukemia/lymphoma. Neurological examination revealed sensory impairment in the distal parts of the extremities with loss of deep tendon reflexes. Nerve conduction study showed motor and sensory demyelinating polyneuropathy, indicating a diagnosis of HTLV-1-associated demyelinating neuropathy. Corticosteroid therapy followed by intravenous immunoglobulin therapy improved his symptoms. Since demyelinating neuropathy associated with HTLV-1 infection is not well recognized, we here report its characteristics and clinical course through our case report and literature review.

Preventing relapse of myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD) with steroids and immunosuppressants is sometimes difficult. There is no standard treatment for refractory cases. We present the case of a 17-year-old female patient with longitudinally extensive myelitis, asymptomatic bilateral optic neuritis, and positive serum MOG-IgG. While taking steroids and several immunosuppressants during the following 14 months, she suffered from two symptomatic relapses in the cerebrum and spinal cord, and multiple asymptomatic relapses in the cerebrum. The patient was negative for MOG-IgG at the second relapse of myelitis. Subcutaneous ofatumumab has suppressed relapse for 13 months. Ofatumumab can be considered a therapeutic option for refractory MOGAD.

Objective

Posterior circulation ischaemic strokes (PCIs) are a clinical syndrome associated with ischemia related to stenosis, in situ thrombosis, or embolic occlusion of the posterior circulation and differ from anterior circulation ischaemic strokes (ACIs) in many aspects. In this study, ACIs and PCIs were evaluated in terms of clinico-radiological and demographic aspects, and the relevance of objective scales to early disability and mortality was investigated.

Methods

The definition of ACIS or PCIS was classified according to the Oxfordshire Community Stroke Project (OCSP). There are mainly two groups divided into ACIs and PCIs. ACIs were included as total anterior circulation syndrome (TACS), partial anterior circulation syndrome (PACS) (right and left), and lacunar syndrome (LACS) (right and left), and PCIs were posterior circulation syndrome (POCS) (right and left). Arrival NIH Stroke Scale/Score (NIHSS) and Glasgow Coma Scale (GCS) scores were evaluated in clinical assessment and modified SOAR Score for Stroke (mSOAR) was for early mortality-based scale prediction. All data were compared, and mean, IQR (if applicable) values and ROC curve analysis were determined.

Results

A total of 100 AIS patients, 50 of whom were ACIs and 50 were PCIs, were included in the study and were evaluated within the first 24 h. Hypertension was the most common disease for both groups. The second most common was hyperlipidemia (82%) in the ACIs and diabetes mellitus (40%) in the PCIs. The frequency of right hemisphere ischemia was higher in ACIs (63.6%) and PCIs (48%). The mean NIHSS and GCS score (also median IQR) was higher in the right ACIs and the highest NIHSS mean was in the right partial anterior circulation syndrome (PACS) (respectively median (IQR): 9.5 (13) and median (IQR):14.5 (3)). The mean NIHSS and GCS score of bilateral posterior circulation syndrome (POCS) were the highest in PCIs (median (IQR):3 (17), (IQR):15 (4) respectively). The mSOAR mean was the highest in the right PACS in ACIs (median (IQR):2.5 (2)) and in bilateral POCs among PCIs (median(IQR):2(2)).

Conclusion

The association of PCIs with hyperlipidemia and the male gender was interpreted, and anterior infarcts were found to cause higher early clinical disability scores. The NIHSS scale was effective and reliable, especially in anterior acute strokes, but also emphasized the necessity of using the GCS assessment together in the first 24 h in the assessment of PCIs. mSOAR scale is a helpful predictor in estimating early mortality not only in ACIs but also in PCIs, similar to GCS.