Posterior reversible encephalopathy syndrome and COVID-19: A series of 6 cases from Lombardy, Italy.

Posterior reversible encephalopathy cases are increasingly being reported in patients affected by COVID-19, but the largest series so far only includes 4 patients. We present a series of 6 patients diagnosed with PRES during COVID-19 hospitalized in 5 Centers in Lombardia, Italy. 5 out of the 6 patients required intensive care assistence and seizures developed at weaning from assisted ventilation. 3 out of 6 patients underwent cerebrospinal fluid analysis which was normal in all cases, with negative PCR for Sars-CoV-2 genome search. PRES occurrence may be less rare than supposed in COVID-19 patients and a high suspicion index is warranted for prompt diagnosis and treatment.

Agenesis of the right internal carotid artery and Klippel-Feil syndrome: case report.

STUDY DESIGN: Case report. OBJECTIVE: To describe the case of a Klippel-Feil anomaly associated with carotid agenesis. SUMMARY OF BACKGROUND DATA: Klippel-Feil anomaly is a spinal malformation characterized by fusion of the cervical vertebrae. Four subtypes have been identified for this congenital disorder with different severity of vertebral fusion and different extra-axial anomalies. Most cases are sporadic, although autosomal dominant and autosomal recessive cases are recognized. It can cause neurologic disorders and is associated to vascular abnormalities. However, agenesis of internal carotid and Klippel-Feil syndrome is an unusual association. METHODS: A 49-year-old woman came to our attention for recurrent transitory ischemic attacks presenting with weakness of left limbs associated with sensory abnormalities. Neurologic examination revealed mild left limb weakness and tactile hypoesthesia. RESULTS: Brain magnetic resonance (MR) and MR angiography demonstrated absence of the right internal carotid and the middle right cerebral artery was filled from the basilar artery. Fusion of vertebral bodies was documented at MR and confirmed at spinal CT scan. The day after the admission the neurologic examination became normal. Ticlopidine was then started. CONCLUSION: Literature of vascular abnormalities in association with Klippel-Feil syndrome takes the form of anecdotal reports. Aortic coarctation, vertebral artery dissection, aneurysms, persistent trigeminal artery, and abnormal origin of internal carotid are described. An unusual association of carotid internal agenesis and Klippel-Feil syndrome is reported with a literature review.

Survival of 793 patients with amyotrophic lateral sclerosis diagnosed over a 28-year period.

We reviewed retrospectively 793 consecutive patients discharged with ALS diagnosis between 1971 and 1998 to identify survival predictors. Vital status in July 2001 was known for all patients. Mean age at onset was 56 (SD 11.7) years and mean age at diagnosis was 57.4 (SD 11.5) years. Onset symptoms were bulbar in 20.8% of patients. The median survival time from symptoms onset was 2.9 years (95% CI 2.7-3.1). Survival from onset was 93% after one, 48% after three and 24% after five years. Median survival was shorter in patients with time between onset and diagnosis <12 months than in those diagnosed> 23 months; (2.1 years vs. 5.9 years; P<0.001). Patients with onset after age 65 had a 4.2 times greater risk of death than patients less than 40 years. Bulbar onset was associated with 1.4 times greater risk of death than spinal onset. Patients diagnosed after 1990 had longer median survival (3.3 years) than those diagnosed in 1971-1979 (2.4 years) (P<0.001). As expected, age, bulbar onset and short time from onset to diagnosis were independent predictors of survival. We also found a significant increase in survival over time, probably due to improved comprehensive treatment of ALS patients in Italy.

Thin section MR study of the basal ganglia in the differential diagnosis between striatonigral degeneration and Parkinson disease.

PURPOSE: Signal abnormalities within the putamen in MRI have been related to tissue degeneration in the striatonigral variant of multiple system atrophy (MSA-P). While previous work demonstrated the high specificity of these MR findings, sensitivity rates were unsatisfactory. We evaluated the specificity and sensitivity of an acquisition protocol using thin section MRI to differentiate MSA-P from Parkinson disease (PD). METHOD: Axial 3-mm-thick conventional T2 and proton density spin echo images at the level of basal ganglia were acquired at 1.5 T in 24 patients with MSA-P and 27 patients with PD. RESULTS: We found an abnormal putaminal T2 hypointensity in 21 of 24 MSA-P patients (87.5% sensitivity) and a proton density hyperintensity in 20 of 24 MSA-P patients (83.3% sensitivity). Three among 27 PD patients had an abnormal putaminal T2 hypointensity (88.8% specificity) and there were no proton density abnormalities (100% specificity). CONCLUSION: Our thin section conventional spin echo protocol showed a substantial increase in MR sensitivity compared with previous reports. We believe that a better depiction of even mild signs of degeneration in the putamen may allow a more widespread use of this technique in the differential diagnosis of parkinsonisms.