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Introduction Nonketotic hyperglycemic hyperosmolar state (NKHHS) is associated with a wide spectrum of neurological syndromes including acute stroke-like deficits. Clinical features and etiology have not been established yet. Methods Here we provide a case illustration and systematic review on non-epileptic acute neurological deficits in NKHSS. The systematic literature search followed PRISMA guidelines and a predefined protocol, including cases of NKHSS with acute stroke-like presentation. Results The database search yielded 18 cases. Hemianopia was the most common clinical presentation (73%), followed by partial or total anterior circulation syndrome (26%). Patients with symptoms of acute anterior circulation infarct were significantly older (69.5 ± 5.1 vs. 52.2 ± 13.9 years; p = 0.03) and showed higher mean glucose levels at the admission vs. those with hemianopia (674.8 ± 197.2 vs. 529.4 ± 190.8 mg/dL; p = 0.16). Brain MRI was performed in 89% of patients, resulting abnormal in 71% of them, especially hemianopic (91%). Subcortical hypointensities in T2-FLAIR MR sequences were present in all the analyzed cases. Cortical DWI hyperintensities were also common (64%). EEG showed diffuse or focal slow wave activity in 68% of patients, especially with visual hallucinations (85%). Neurological symptoms completely resolved in 78% of patients within 6 (IQR 3–10) days, following aggressive treatment and glucose normalization. Conclusions Our results suggest neuronal dysfunction on a metabolic basis as the leading cause of acute neurological deficits in NKHHS. Despite the generally favorable prognosis, prompt identification and aggressive treatment are crucial to avoid irreversible damage. Larger cohort studies are needed to confirm our findings.

Increased incidence rates of amyotrophic lateral sclerosis (ALS) have been recently reported across various Western countries, although geographic and temporal variations in terms of incidence, clinical features and genetics are not fully elucidated. This study aimed to describe demographic, clinical feature and genotype–phenotype correlations of ALS cases over the last decade in the Emilia Romagna Region (ERR). From 2009 to 2019, our prospective population-based registry of ALS in the ERR of Northern Italy recorded 1613 patients receiving a diagnosis of ALS. The age- and sex-adjusted incidence rate was 3.13/100,000 population (M/F ratio: 1.21). The mean age at onset was 67.01 years; women, bulbar and respiratory phenotypes were associated with an older age, while C9orf72-mutated patients were generally younger. After peaking at 70–75 years, incidence rates, among women only, showed a bimodal distribution with a second slight increase after reaching 90 years of age. Familial cases comprised 12%, of which one quarter could be attributed to an ALS-related mutation. More than 70% of C9orf72-expanded patients had a family history of ALS/fronto-temporal dementia (FTD); 22.58% of patients with FTD at diagnosis had C9orf72 expansion (OR 6.34, p = 0.004). In addition to a high ALS incidence suggesting exhaustiveness of case ascertainment, this study highlights interesting phenotype–genotype correlations in the ALS population of ERR.

A healthy 53-year-old man developed acute flaccid paraplegia, spinal sensory level, and urinary retention. Examination revealed bilateral pronator drift. MRI demonstrated demyelinating non-enhancing lesions involving corticospinal tracts (CSTs), middle cerebellar peduncles, and spinal cord (figure 1). CSF analysis disclosed lymphocytic pleocytosis and mildly elevated protein. Euroimmun fixed cell-based assay for serum anti–myelin oligodendrocyte glycoprotein (MOG) and anti–aquaporin-4 (AQP4) antibodies showed low positivity for MOG immunoglobulin G (IgG) (1:10). Treatment with high-dose glucocorticoids and IV immunoglobulin was effective (figure 2). Long CST lesions were found in anti–AQP4 IgG–seropositive neuromyelitis optica spectrum disorders.1 Bilateral extensive lesions following CST are a novel finding in MOG-related CNS autoimmunity.2

Headache is a common Emergency Department (ED) problem accounting for 1.7%–4.5% of patients every year. The main aim in the ED is to differentiate between primary and secondary headache. Although secondary headache represents the lowest percentage (20%) of ED patients presenting with headache, it can be life threatening. Patients with primary headache require a follow-up programme on discharge from the ED with a specialist headache clinic taking responsibility for these patients. This procedure was applied at Ravenna Hospital ED for 2 years and recorded (unpublished data) a reduction in the number of repeat visits to the ED for headache and the number of inappropriate admissions for headache (DRG 25).