Background: Methods to assess impaired consciousness in acute stroke typically include the Glasgow Coma Scale (GCS), but the verbal component has limitations in aphasic or intubated patients. The FOUR (Full Outline of UnResponsiveness) score, a new coma scale, evaluates 4 components: eye and motor responses, brainstem reflexes and respiration. We aimed to study the interobserver variability of the FOUR score in acute stroke patients. Methods: We prospectively enrolled consecutive patients with acute stroke admitted from February to July 2008 to the stroke unit of our Neurology Department. Patients were evaluated by neurology residents and nurses using the FOUR score and the GCS. For both scales, we obtained paired and total weighted kappa values (Kw) and intraclass correlation coefficients (ICC). NIH stroke scale was also recorded on admission. Results: We obtained a total of 75 paired evaluations in 60 patients (41 cerebral infarctions, 15 cerebral hemorrhages and 4 transient ischemic attacks). Thirty-three (55%) patients were alert, 17 (28.3%) drowsy and 10 (16.7%) stuporous or comatose. The overall rater agreement was excellent in the FOUR score (Kw 0.93; 95% CI 0.89–0.97) with an ICC of 0.94 (95% CI 0.91–0.96) and in the GCS (Kw 0.96; 95% CI 0.94–0.98) with an ICC of 0.96 (95% CI 0.93–0.97). A good correlation was found between the FOUR score and the GCS (ρ 0.83; p < 0.01) and between the FOUR score and the NIH stroke scale (ρ –0.78; p < 0.001). Conclusions: The FOUR score is a reliable scale for evaluating the level of consciousness in acute stroke patients, showing a good correlation with the GCS and the NIH stroke scale.

Methotrexate (MTX) is considered the main agent for the treatment of rheumatoid arthritis (RA). Neurotoxicity is often mild, but severe encephalopathy can develop, especially with intrathecal or intravenous administration. In rare cases, this syndrome has been observed in patients on long-term low-dose oral administration. A 68-year-old male was diagnosed with RA and on treatment with oral MTX 25 mg weekly for 4 years. The patient started with progressive dysarthria, ataxia and cognitive dysfunction. Complementary tests were normal. Magnetic resonance imaging (MRI) showed hyperintense lesions in both cerebellar hemispheres on T2-weighted and FLAIR images with a diffusion restriction on diffusion-weighted imaging (DWI) and on the apparent diffusion coefficient map (ADC). On postgadolinium T1-weighted images, there were mild enhancements. Spectroscopy showed a demyelinating pattern. A pharmacogenetics determination was made, showing a heterozygous genotype in the MTHFR and ABCB1 genes. Medication with antirheumatic drug was stopped immediately on admission, and the patient gradually improved. MTX-induced leukoencephalopathy can occur even with low-dose administration. The exact pathogenic mechanism is still unknown, but it is hypothesised that it could be the result of a cumulative toxic effect on the blood-brain barrier. The nature of the relationship between the polymorphism and CNS toxicity is still unclear, and thus, further studies are warranted. Often located in the occipital lobes, the involvement of the cerebellum is quite rare. Early recognition of the condition and withdrawal of the drug lead to a better prognosis.

We emphasize the diagnostic importance of the biopsy in cases of chronic pachymeningitis and stress that diverse entities can cause progressive supranuclear palsy-like phenotypes.

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical radiological syndrome, characterised by acute headache, altered consciousness, seizures and hypertension. The most frequent causes are hypertensive encephalopathy, eclampsia and some immunosuppressive therapies. The pathogenesis remains unclear, but it appears to be related to altered cerebral circulation, producing oedema that can be seen on MRI, and it resolves in 2 or 3 weeks. In the present report, a possible first reported case of cyclophosphamide-induced RPLS in a 27-year-old man with high blood pressure (HBP) and glomerulonephritis caused by Goodpasture syndrome, treated with cyclophosphamide during the last month and prednisone for glomerulonephritis resulting from Goodpasture syndrome without other immunosuppressive drugs, is described.Symptoms appeared during a hypertensive crisis, but when cyclophosphamide was replaced by rituximab and hypertension was controlled, the patient did not have neurological symptoms. Almost all reported cases induced by immunosuppressive therapy or other causes were associated with hypertension as well.