Early cortical atrophy in REM sleep behavior disorder. / Atrofia cortical precoz en el trastorno de conducta del sueño REM.

INTRODUCTION: The presence of cortical atrophy (focal or diffuse) prior to the development of symptoms of cognitive impairment could predict the earliest cases of neurodegenerative disease in patients with REM sleep behavior disorder (RSBD). We reviewed the usefulness of cranial CT and MRI as early markers of cortical atrophy in patients with RSBD at our center. PATIENTS AND METHODS: Retrospective observational descriptive analysis of patients diagnosed with RSBD from October 2012 to October 2022. All with cranial CT or MRI, evaluated by a neuroradiologist. RESULTS: 54 patients were included, 21 women (38.88%), 33 men (61.12%), mean age at diagnosis of RSBD: 69.04±12.625 years. Of the 54 patients, 44 (81.48%) had imaging tests consistent with their age, and 10 had atrophy greater than expected for their age. Of the 54 patients, 21 (38.88%) with a diagnosis of neurodegenerative disease, 33 (61.12%) persist as idiopathic, almost all with more than 5years of evolution (range of 1 to 10years of evolution without diagnosis). Of the 10 (18.52%) patients with greater atrophy, all were diagnosed with neurodegenerative disease (8 in 1year, 2 in 8years). CONCLUSIONS: Almost half of our series have developed a neurodegenerative disease in the first 10years of evolution. The majority of them presented global cortical atrophy measured by the GCA scale in the first year of diagnosis, without other neurological symptoms. Patients who did not show cortical atrophy at diagnosis have not yet developed the neurodegenerative disease in 10years of evolution. In our experience, the absence of cortical atrophy on cranial MRI or CT (measured by scales such as GCA) at the diagnosis of RSBD seems to predict slower progression cases. These data should be corroborated with larger series.

Howl: Nightmare or REM Sleep Behavior Disorder?

Objective: Nightmare disorder consists of the appearance of unpleasant and vivid, repeated dreams, with a situation of discomfort and anguish on awakening. Its prevalence is 3%-4% in adults. They do not associate muscle mobilization during this phase. REM sleep behavior disorder (RSBD) is a rare parasomnia (0.5% of people older than 60 years of age), characterized by the presence of unpleasant dreams, with violent content, and vigorous movements of limbs (kicks and punches), reflecting a loss of muscle atony typical of the REM phase of sleep. Language (screams and words) can also be emitted. The same clinical manifestations of RSBD can appear in other sleep disorders. The diagnosis requires the performance of a polysomnography. Methods: We present the case of a 41-year-old man referred for vivid and unpleasant dreams, beginning in the last year, related to work stress. Results: The polysomnography showed the loss of atony in the REM phase and emission of a prolonged howl after which the patient continues in the REM phase. Discussion: Prolonged howling is a very rare symptom in sleep disorders, and very atypical in RSBD, so polysomnography is essential to confirm the diagnosis and rule out other parasomnias.

Anxiety and depression in patients with narcolepsy.

We analysed the co-existence of psychopathology in patients with narcolepsy at our centre. We performed an observational retrospective descriptive analysis of patients with a diagnosis of narcolepsy, with and without psychopathology, who attended our sleep disorders unit from October 2012 to October 2021. A total of 51patients with narcolepsy (mean [SD] age 41.10 [14.71] years; 23 [45.1%] males and 28 [54.90%] females) were included. In all, 27 patients (52.94%) and 24 patients (47.06%) had narcolepsy with and without cataplexy, respectively. Of the total, 18 (33.33%) had a mood disorder: 18 with anxiety disorder (33.33%). Of these patients 14 (27.45%) had major depression, two (4%) had attempted suicide, one (2%) had manic outbreak, and one (2%) had substance abuse. Of the 18 patients with anxiety and depression, 10 (55.55%) and eight (44.44%) had narcolepsy with and without cataplexy, respectively. In the comparative analysis, a statistically significant relationship was found between younger age and the presence of anxiety. The prevalence of anxiety and depression in patients with narcolepsy was triple that of the general population, especially in younger patients. Psychopathology precedes the diagnosis of narcolepsy in most patients, not being reactive to diagnosis. This high prevalence suggests a possible biological relationship between both disorders, which should be assessed with larger studies.

Strongyloides hyperinfection syndrome due to corticosteroid therapy after resection of meningioma: illustrative case.

BACKGROUND: Strongyloidiasis is an underdiagnosed and preventable life-threatening disease caused by infection with the helminth Strongyloides stercoralis. Chronic asymptomatic infection can be sustained for decades, and immunosuppression can lead to disseminated infection, with a mortality rate of 70%-100%. In the neurosurgical population, corticosteroids are the most consistent cause of hyperinfection. OBSERVATIONS: The authors present the case of a 33-year-old woman of Paraguayan origin who was diagnosed with sphenoid planum meningioma and treated with a high dose of corticosteroids on the basis of the diagnosis. She underwent surgery, and pathological anatomy reflected grade I meningioma. After the surgery, she started with a history of dyspnea, productive cough, fever, and urticarial rash. Later, she presented with intestinal pseudo-obstruction and bacterial meningitis with hydrocephalus. Serology was positive for Strongyloides (enzyme-linked immunosorbent assay), and she was diagnosed with hyperinfection syndrome. Ivermectin 200 µg/kg daily was established. LESSONS: It may be of interest to rule out a chronic Strongyloides infection in patients from risk areas (immigrants or those returning from recent trips) before starting treatment with corticosteroids.

Encephalopathy in severe SARS-CoV2 infection: Inflammatory or infectious?

Concerning the letter by Moriguchi et al., we describe our experience with a case of encephalopathy with and atypical damage on magnetic resonance imaging (MRI) in a patient with severe infection due to the SARS-CoV2 virus. A 56-year-old woman, without previous pathologies, developed cough, fever, and respiratory failure for five days, after returning from a 6-day trip to Venice. Chest radiography shows a large bilateral interstitial infiltrate. In the first 24 hours, she was admitted to the Intensive Care Unit (ICU) for severe respiratory failure and positive protein chain reaction-PCR in nasal exudate. She needed intubation for ten days. In the first 48 hours outside the ICU, she developed an acute confusional syndrome (hyperactive delirium). Neurological examination showed temporal-spatial disorientation and incoherent fluent speech. An electroencephalogram (EEG) showed generalized hypovoltaic activity. Cranial magnetic resonance imaging showed a bilateral and symmetrical increase in the supratentorial white matter's signal intensity, with a discrete thickening of both temporal lobes, with a slight increase in signal intensity and a sequence of normal diffusion. The lumbar puncture showed no changes (glucose 71 mg/dL, protein 30 mg/dL, 1 leukocyte). Within 72 hours of starting symptoms, she was neurologically asymptomatic. Our final diagnosis was an inflammatory encephalopathy related to a SARS-CoV2 infection.

Rheumatoid meningitis mimicking progressive supranuclear palsy.

INTRODUCTION: Rheumatoid meningitis is an uncommon manifestation of longstanding rheumatoid arthritis and few cases have been described. The clinical presentation is extremely variable as reported in medical literature. CASE REPORT: We report a 71-year-old woman with 15 years of seropositive rheumatoid arthritis who developed neurological complications: cognitive deterioration; hypomimia; limitation on vertical gaze; and axial stiffness, resembling progressive supranuclear palsy and seizures. Brain magnetic resonance imaging showed a diffuse dural plaque on both frontal and temporal lobes exhibiting homogeneous gadolinium enhancement. There was diffuse leptomeningeal enhancement and hyperintense white matter lesions. The final diagnosis made by image-guided biopsy showed rheumatoid pachymeningitis. After the definitive diagnosis, high doses of corticosteroids and immunosuppressive treatment were started. CONCLUSIONS: 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.

Young women have poorer outcomes than men after stroke.

BACKGROUND AND PURPOSE: Gender differences in stroke outcome have not been fully assessed in young patients. METHODS: We conducted an observational study of consecutive young ischemic stroke patients (≤ 50 years of age) admitted to a stroke unit (January 1999 to December 2009). Basal data, subtype of ischemic stroke, stroke severity [Canadian Neurological Scale (CNS)], length of hospital stay, inhospital complications, mortality and functional outcome at discharge [modified Rankin Scale (mRS) score] were analyzed. For stroke severity and outcome analyses, 2 age groups were established: 15-30 (very young group) and 31-50 years old (middle-aged young group). RESULTS: A total of 310 patients were enrolled; 128 females and 182 males. The mean age was similar in women and men (41.07 ± 8.6 vs. 42.12 ± 8.2, NS). Migraine was more frequent in women, whereas arterial hypertension, hyperlipidemia, alcohol abuse, current smoking and atherothrombotic infarction were more frequent in men (p < 0.05). Females presented greater stroke severity than men [median CNS (IQR) = 8 (3.5) vs. 9 (2.5), p = 0.014] except in the very young group [median CNS (IQR) = 9 (1.8) vs. 8 (5), p = 0.022]. Female sex was a predictor of unfavorable outcomes (mRS >2) at discharge in the total sample (OR = 3.33; 95% CI = 1.41-7.84) and in the middle-aged young group (OR = 2.62; 95% CI = 1.05-6.53), adjusted by baseline data, stroke subtype, inhospital complications, length of stay and stroke severity. CONCLUSIONS: Female gender is associated with worse outcomes in adult ischemic stroke patients up to 50 years old. However, this effect is not observed in younger patients (15-30 years).

Cyclophosphamide-induced reversible posterior leukoencephalopathy syndrome.

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.