eNeurologicalSci最新文献

Purpose

To analyze changes in peripapillary retinal nerve fiber layer (RNFL) and ganglion cell complex (GCC) thickness in migraine patients with and without aura compared to healthy controls and to identify factors influencing the occurrence of these anomalies.

Methods

This is a cross-sectional case-control study including migraine patients and control subjects. All patients and controls underwent a complete ophthalmological examination, RNFL and GCC thickness measurements using a spectral domain-OCT device.

The duration of migraine, the frequency and duration of migraine attacks, the migraine disability assessment (MIDAS) and migraine severity scale (MIGSEV) questionnaire scores were recorded.

Results

One hundred and twenty eyes from 60 patients (60 eyes in the migraine without aura (MWoA) group and 60 eyes in the migraine with aura (MWA) group) were included. Control group included 30 age and gender matched healthy participants (60 eyes). OCT revealed that RNFL and GCC thickness were significantly reduced in the migraine without aura (MWoA) and in the migraine with aura (MWA) groups compared to the control group and in the migraine with aura (MWA) group compared to the migraine without aura (MWoA) group. Prolonged disease duration was associated to decreased GCC thickness. RNFL and GCC thickness were correlated to disease severity, attack frequency and duration. In the multivariate study, duration of migraine and attack frequency were the main determinant factors of nasal GCC thickness. Disease severity was the main determinant of RNFL and GCC thickness, with the exception of the nasal sector.

Conclusion

Our study emphasize the significant impact of both types of migraine on retinal structures. OCT would serve as a valuable biomarker in migraine.

A 72-year-old man presented with a 6-month history of decreased voluntary speech. Sparse speech and decreased word fluency were observed. Articulation, naming, comprehension, and repetition were preserved. Agrammatism and paraphasia were not observed. These characteristics matched those reported as dynamic aphasia. Other findings were mild behavioral symptoms, recent memory impairment, and right hemiparkinsonism. The patient‘s voluntary speech continued to reduce and behavioral symptoms progressed. Brain MRI including voxel-based morphometric analysis showed left-dominant white matter volume reduction in the frontal lobe including those between the left supplementary motor area (SMA)/preSMA and the frontal operculum, likely involving the frontal aslant tract (FAT). The patient became completely mute after two years from disease onset and died of aspiration pneumonia. The neuropathological diagnosis was corticobasal degeneration (CBD). This case suggests that dynamic aphasia may be the initial sign of CBD and that early involvement of left FAT may be responsible for this feature.

Body lateropulsion (BLP) has been reported several times after cerebellar infarction. It is usually ipsilateral to the cerebellar infarction, particularly when limited to the rostral cerebellum. In contrast, contralesional BLP after cerebellar infarction has been reported in more caudal regions of the cerebellum (such as the nodulus or the tonsil).

We report the case of a small infarction of the left anterior paravermis of the rostral cerebellum which resulted in bilateral symptoms: ipsilesional limb ataxia and, unexpectedly, contralesional BLP.

Several neurological pathways were potentially involved. Both right and left dorsal spinocerebellar tracts may have been damaged by the infarction of the left anterior paravermis. On the other hand, the proximity of the infarct to the superior cerebellar peduncle may have caused damage to the vestibular pathways (fastigio-vestibular or dentato-vestibular tracts), as they exit the cerebellum by the superior cerebellar peduncle. A lesion of the cerebellum close to the superior cerebellar peduncle could result in a contralesional BLP.

Introduction

Infarction of the artery of Percheron (AOP) is a rare vascular condition where a single arterial branch supplies blood to the thalamic and midbrain regions, leading to neurological deficits. The challenge lies in its often-delayed diagnosis due to its rarity and diverse clinical presentations, necessitating heightened awareness among clinicians for expedited diagnosis and appropriate therapeutic interventions.

Materials and methods

All relevant studies involving patients diagnosed with infarction of AOP were retrieved from PubMed, Google Scholar, Web of Science, and Scopus. Only human studies that were published in full English-language reports were included. Included in the search were the terms “Artery of Percheron,” “infarction,” “stroke,” and “demarcation”. Age, gender, presenting symptoms, treatment, recovery time, and outcome of patients with AOP infarction were all recorded.

Results

A systematic review was conducted on a total of 530 articles, out of which 130 articles met the specified requirements. The average age is 59, with men comprising 57.7% of the population. The symptoms reported were visual disturbance in 43.9% of cases and changed mental state in 77.2% of cases. Treatment options include conservative management (85.4%), thrombolysis (11.3%), and other approaches. The optimal age range for recovery is between 41 and 50 years old.

Conclusion

Our study on acute AOP infarction highlights male predominance, common comorbidities like hypertension and diabetes, and prevalent symptoms including visual disturbance and altered mental state. Early recognition is crucial, with thrombolytic therapy within the critical time window showing promising outcomes. These findings offer insights for enhanced clinical management of AOP infarction.

Familial paroxysmal non-kinesigenic dyskinesia, which is a major form of paroxysmal dyskinesias, is characterized by intermittent attacks that include one side, subsequently spreading to the other side, involving the limbs and face, and is triggered by caffeine, alcohol, emotional stress, fatigue, and sleep deprivation, but not by sudden movement. A 26-year-old man had experienced dystonic movements and a choreiform right arm spreading to his arms, legs, and face since the age of one year. Oral dyskinesias and, rarely, dysarthria were also observed. Attacks lasting approximately five minutes occurred several times per day. Over three generations, his family members inherited a c.26C > T (p. Ala9Val) missense mutation in exon 1 of PNKD/MR-1 in an autosomal dominant manner and reported similar symptoms with clinical manifestations ranging from mild to severe. His scores on the Self-Rating Depression Scale, State–Trait Anxiety Inventory, and Profile of Mood States were high. This suggests that the patient also had comorbidities of anxiety and depression. The patient's attacks decreased from two times per week to once every two months, and his State–Trait Anxiety Inventory score decreased by 5–10 points on treatment with clonazepam and sertraline, allowing his condition to become stable enough that he was able to participate in society. Drug therapy with clonazepam and sertraline is the preferred treatment for reducing attacks in PNKD patients with strong anxiety and depression.

Objective

We present the fifth case of candida dubliniensis meningitis in a young immunocompetent host and suggest extracorporeal membrane oxygenation (ECMO) as a potential risk factor for colonization.

Methods

A 22-year-old immunocompetent female presented with a diagnosis of bacterial meningitis. Two years prior, she received ECMO for Covid-19 pneumonia complicated by viral myocarditis & Takutsobo cardiomyopathy. Following discharge, she reported headaches of increasing intensity, all refractory to treatments. Brain magnetic resonance imaging (MRI) was inconclusive. Two weeks prior to her presentation, she was admitted for worsening headaches with cranial nerve VI palsy. Lumbar puncture (LP) revealed white blood cell count (WBC) of 166 cells/μL with neutrophilic predominance and her symptoms progressed, despite 5 days of treatment with broad spectrum antibiotics. All cultures returned negative.

Results

At her current presentation, repeat LP revealed 835 WBC/mm3, 225 mg/dL protein, and 4 mg/100 mL glucose. Brain MRI revealed nodular enhancement in the brainstem and communicating hydrocephalus. MRI of the lumbar spine revealed meningeal enhancement. Cerebrospinal fluid (CSF) cultures came back positive for C.dubliniensis. Treatment began with Amphotericin B and Flucytosine.

Discussion

When clinical suspicion for fungal meningitis is high, repeate LP and CSF analysis is indicated to establish a definitive diagnosis and begin treatment. Additional studies are needed to confirm risk factors, like ECMO, for the colonization of C.dubliniensis, which likely predisposes individuals to invasive candidiasis.

Background

Epilepsy remains a significant public health concern in Sub-Saharan Africa (SSA) where diverse etiological factors contribute to its prevalence. Among these factors are conditions originating from the neuroectoderm, such as tuberous sclerosis. Insufficient medical attention and a lack of comprehensive multidisciplinary care contribute to its under-recognition.

Materials and methods

We conducted a retrospective descriptive study, involving 12 patients admitted to the neurology and pediatric departments of the University Hospital Ignace Deen between 2010 and 2022 due to recurring epileptic seizures. Subsequently, these patients were diagnosed with Tuberous sclerosis using the Schwartz 2007 criteria. The aim of this study is to reassess this condition from a clinical and paraclinical point of view in a tropical environment.

Results

Tuberous sclerosis, also known as Bourneville disease, was diagnosed in 12 patients exhibiting focal motor seizures and complex focal seizures likely associated with cortical and subcortical tubers detectable by EEG and neuroimaging, including CT and MRI. Delayed treatment resulted in varying degrees of mental decline. Additionally, some patients displayed cardiac hamartomas and intracranial posterior and anterior aneurysms as minor diagnostic indicators.

Conclusion

The study reveals a consistent clinical presentation accompanied by deteriorating neurological and psychological symptoms attributed to delayed multidisciplinary management. These findings are utilized to assess therapeutic strategies and prognostic outcomes.

Although the impact of the first wave of the COVID-19 pandemic on people with several neurological diseases has been largely investigated, little is available concerning people with Huntington's disease (HD).

The main objective of the study was to interview people with HD and their caregivers in the Auvergne-Rhone Alpes region, France.

The interview consisted of 16 items concerning general and medical information, and the impact of the first wave of COVID-19 pandemic on the medical care of people with HD and on their caregivers. The questionnaire was made available as online survey from October 1st, 2020 until November 15th, 2020.

Fifty-two subjects participated (13 men, 39 women, mean age of 47.3 ± 15.5 years). Almost half participants (48%) experienced a worsening of pre-existing symptoms, with new-onset symptoms in the 44% of cases. The most frequent worsening was reported in gait and balance issues (67%), fatigue (58%), anxiety (50%), and depression (50%). The 70.8% of participants reported an inappropriate overall care of HD due to long delays to access medical care (30%) and other health care teams (60%). More than half of the participants (54.2%) reported that the COVID-19 pandemic had a negative impact on their caregiver/family.

Our findings emphasize the negative impact of the first wave of COVID-19 pandemic on the healthcare of HD population and their caregivers. Not only some symptoms were aggravated, but new symptoms appeared during the pandemic. In the future, health policies should be considered to improve the care of patients with rare diseases such as HD.

Background

Spontaneous subarachnoid hemorrhage (sSAH) is a medicosurgical emergency with high morbidity and mortality. The aimed of this study was to describe the clinical features and outcome of sSAH in Cameroon.

Methods

We reviewed medical records of patients aged ≥15 years old, admitted for sSAH from Januray 2011 to December 2020 in the Douala General Hospital. The diagnosis of sSAH was confirmed by neuroimaging (CT scan or MRI). Clinical and radiological severities were assessed by the WFNS score and the modified Fisher score respectively. Factors associated to in-hospital mortality was identified using cross-table (RR and 95%CI).

Results

Among the 111 cases of sSAH reviewed in emergencies records, we included 70 patients. The mean age was of 55.6 ± 13.6 years. Female were predominant (57.1%). Altered consciousness was the main clinical feature (55.7%). The WFNS score was grade 4–5 in 54.3% of patients. And 75.7% of cases presented a modified Fisher score of 3–4. Ruptured of intracranial aneurysm was the most common etiology (46.2%). Endovascular treatment and/or surgical treatment were not avaible. Hospital-based mortality was 40% and factor associated with death were Altered consciousness (RR: 4.3, 95%CI:1.52–12.33, p = 0.004), coma (RR: 23.9, 95%CI:2.85–200.62, p = 0.004), WFNS grade 5 (RR: 18.2, 95%CI:3.7–92.3, p < 0.001), and hospital length ≤ 7 days (RR: 13.5, 95%CI:4.28–42.56, p < 0.001).

Conclusion

Mortality and disability of sSAH are still high in our setting. Further studies with prospective follow up of patients are needed to determine the long-term outcome of these patients.