Posterior reversible encephalopathy (PRES) is a rare occurrence in patients with Guillain–Barré syndrome (GBS) with only nine adult cases reported to date. We conducted a review of the literature and compared previous cases with a novel case admitted to our clinic. In light of the timing of the symptoms, it is assumable that arterial hypertension can develop acutely during a phase of GBS-related autonomic dysfunction and subsequently precipitates PRES. According to this, dysautonomia caused by GBS could precede motor weakness; thus, PRES in the absence of any strong alternative etiology may suggest an underlying GBS.

Evidences from either small series or spontaneous reporting are accumulating that SARS-CoV-2 involves the Nervous Systems. The aim of this study is to provide an extensive overview on the major neurological complications in a large cohort of COVID-19 patients. Retrospective, observational analysis on all COVID-19 patients admitted from February 23rd to April 30th, 2020 to ASST Papa Giovanni XXIII, Bergamo, Italy for whom a neurological consultation/neurophysiological assessment/neuroradiologic investigation was requested. Each identified neurologic complication was then classified into main neurologic categories. Of 1760 COVID-19 patients, 137 presented neurologic manifestations that manifested after COVID-19 symptoms in 98 pts and was the presenting symptom in 39. Neurological manifestations were classified as: (a) cerebrovascular disease [53 pts (38.7%)] including 37 ischemic and 11 haemorrhagic strokes, 4 transient ischemic attacks, 1 cerebral venous thrombosis; (b) peripheral nervous system diseases [31 (22.6%)] including 17 Guillain–Barrè syndromes; (c) altered mental status [49 (35.8%)] including one necrotizing encephalitis and 2 cases with RT-PCR detection of SARS-Cov-2 RNA in CSF; (d) miscellaneous disorders, among whom 2 patients with myelopathy associated with Ab anti-SARS-CoV-2 in CSF. Patients with peripheral nervous system involvement had more frequently severe ARDS compared to patients with cerebrovascular disease (87.1% vs 42%; difference = 45.1% 95% CI 42.0–48.2; χ2= 14.306; p < 0.0002) and with altered mental status (87.1% vs 55.6%; difference = 31.5% 95% CI 27.5–37.5%; χ2= 7.055; p < 0.01). This study confirms that involvement of nervous system is common in SARS-CoV-2 infection and offers clinicians useful information for prevention and prompt identification in order to set the adequate therapeutic strategies.

ObjectiveTo compare the diagnostic value of striatal 123I‐2β‐carbomethoxy‐3β‐(4‐iodophenyl)‐N‐(3‐fluoropropyl) nortropane (123I‐FP‐CIT) single photon emission computed tomography (SPECT) and 123I‐metaiodobenzylguanidine (123I‐MIBG) myocardial scintigraphy in differentiating dementia with Lewy bodies (DLB) from other dementia types.MethodsThis prospective longitudinal study included 30 patients with a clinical diagnosis of DLB and 29 patients with non‐DLB dementia (Alzheimer disease, n = 16; behavioral variant frontotemporal dementia, n = 13). All patients underwent 123I‐FP‐CIT SPECT and 123I‐MIBG myocardial scintigraphy within a few weeks of clinical diagnosis. All diagnoses at each center were agreed upon by the local clinician and an independent expert, both unaware of imaging data, and re‐evaluated after 12 months. Each image was visually classified as either normal or abnormal by 3 independent nuclear physicians blinded to patients' clinical data.ResultsOverall, sensitivity and specificity to DLB were respectively 93% and 100% for 123I‐MIBG myocardial scintigraphy, and 90% and 76% for 123I‐FP‐CIT SPECT. Lower specificity of striatal compared to myocardial imaging was due to decreased 123I‐FP‐CIT uptake in 7 non‐DLB subjects (3 with concomitant parkinsonism) who had normal 123I‐MIBG myocardial uptake. Notably, in our non‐DLB group, myocardial imaging gave no false‐positive readings even in those subjects (n = 7) with concurrent medical illnesses (diabetes and/or heart disease) supposed to potentially interfere with 123I‐MIBG uptake.Interpretation123I‐FP‐CIT SPECT and 123I‐MIBG myocardial scintigraphy have similar sensitivity for detecting DLB, but the latter appears to be more specific for excluding non‐DLB dementias, especially when parkinsonism is the only “core feature” exhibited by the patient. Our data also indicate that the potential confounding effects of diabetes and heart disease on 123I‐MIBG myocardial scintigraphy results might have been overestimated. Ann Neurol 2016;80:368–378

Background: The El Escorial diagnostic criteria are the most commonly used in clinical studies and therapeutic trials in patients with amyotrophic lateral sclerosis (ALS). The accuracy of the El Escorial criteria was tested in clinical practice, but the reliability is unknown when the diagnosis of ALS must be assessed on the basis of medical records. Objective: To assess the reliability of the El Escorial criteria for the diagnosis of ALS in different settings. Design and Methods: Semistructured forms were used to include the main diagnostic information on 20 patients with definite (n = 6), probable (n = 6), possible (n = 6), and suspected ALS (n = 2) and 19 patients with clinical conditions considered in the differential diagnosis. Agreement was tested by comparing the diagnosis made by the attending physician (the ‘gold standard’) with that of 4 raters with different backgrounds: a teaching neurologist with research and practical experience in the field of motor neuron disorders, a neurologist with specific interest in motor neuron disorders and neurophysiological background, a neurophysiologist, and a general neurologist with only occasional ALS patients. Sources of disagreement were discussed and the agreement was tested further on the medical records of 98 additional cases taken from an ongoing ALS registry. Eight additional cases (ALS: 4; other conditions: 4) were examined directly by the 4 raters. Results: The interrater agreement on the medical records was poor (overall kappa 0.05–0.29). When the differential diagnosis was made between ALS (all diagnostic levels) and other conditions, interrater agreement was at best modest, with moderate variations when raters were compared in pairs (kappa 0.03–0.58) and when each rater was compared with the physician (kappa 0.27–0.51). Agreement was higher after direct examination of the patients (kappa 0.33–1) and increased significantly on the medical records after training (overall kappa 0.52–0.79). However, concordance was low (overall kappa 0.08–0.36), even after training, at the lowest diagnostic level (definite to suspected ALS vs. other conditions). Conclusions: The El Escorial criteria are a poor diagnostic indicator when patients’ records are examined. Although medical education significantly improves the reliability of the criteria, concordance is still modest when the diagnosis includes suspected ALS.

INTRODUCTION: As amyotrophic lateral sclerosis (ALS) is a rare neurological disorder, many sources must be investigated for complete case ascertainment. Hospital discharge diagnoses (HDDs) are a source of administrative data for patients hospitalized for a given disease. The accuracy of HDDs was tested, to assess whether they could be used to trace patients with amyotrophic lateral sclerosis (ALS).METHODS: All the HDDs referring to patients with motor neuron disease (MND) (ICD-9 code 335.2) were identified from primary hospital discharge diagnoses made during the years 1994-95 in six target provinces of the Lombardy region in Northern Italy (total population 2 922 920). The medical records of the patients were reviewed and each patient was then accepted or rejected according to whether he/she did or did not satisfy the El Escorial criteria for the diagnosis of ALS. The ALS discharge diagnoses filed in the study period in the neurology departments of all the regional hospitals were the 'gold standard'. Newly diagnosed patients registered during the year 1998 in the same provinces served for the calculation of the incidence of the disease. Sensitivity, specificity and positive predictive value were calculated. Data were presented separately for all the patients and those who were indicated as first hospital admissions.RESULTS. During the study period a total of 1 012 122 hospital discharge records were coded in the six index provinces. The ICD-9 code 335.2 was reported in 408 of these (0.04%). These discharge diagnoses were made in 267 patients, 245 of whom were resident in Lombardy at the time of hospital admission. The hospital records of 237 cases were examined. In 95 of these the medical records showed a diagnosis other than ALS. Other motor neuron diseases were listed for 50 patients. Thirteen additional cases were traced through neurology department files. The sensitivity of the HDDs was 91.6%, the specificity 99.9%, the positive predictive value 65.4%. The corresponding values for first-hospitalized patients were 97.7, 99.9, and 37.3. The overall ALS incidence rate obtained from the HDDs was 2.1 per 100 000 (95% CI 1.3-3.3). The corresponding rate calculated after exclusion of the false positives and inclusion of the false negatives was 0.8 (95% CI 0.1-2.1) and that obtained using the 1998 cases registered in the same areas was 1.6 (95% CI 0.7-2.7).CONCLUSIONS: The incidence of ALS is overestimated when HDDs are used for the estimate. Nevertheless, with improved quality, administrative data could be useful for the estimate of prevalence and incidence.