Cranial Neuropathy As Initial Manifestation of Primary Sjögren's Syndrome: A Case Series With Literature Review.

The clinical spectrum of primary Sjögren's syndrome (PSS) extends beyond its classical manifestations. This work explores an unusual aspect of PSS, namely the initial presentation of cranial neuropathy. The study was conducted over a period of 22 months, from January 2022 to October 2023. Of 58 PSS patients, only five (four women and one man) had cranial neuropathy as their initial manifestation. Only one patient had sixth cranial nerve involvement, three had acute optic neuritis (second cranial nerve), and three had fifth cranial nerve involvement. The diagnosis of PSS was retained according to the 2016 ACR-EULAR criteria. All patients received symptomatic and immunosuppressive treatments. The course was favorable for all patients. The purpose of this case series is to show that cranial neuropathy can be the initial manifestation of PSS, which should be systematically investigated after the elimination of the most common etiologies of cranial neuropathy, particularly in the elderly.

Anti-seizure medication prescription preferences: a Moroccan multicenter study.

Background: The management of epilepsy is mainly based on antiseizure medications (ASMs). More than 20 ASMs have been introduced in clinical practice, providing a multitude of prescription choices. To date, there are no published data on the trends in ASMs prescriptions in Morocco. Therefore, we conducted a survey among practicing neurologists in seven tertiary referral hospitals in Morocco to study the current ASMs prescription preferences and their modifying factors. Methods: Our descriptive and analytical cross-sectional study was based on a survey sent between January and April 2022 to neurologists practicing in seven tertiary referral hospitals in Morocco. Information regarding the prescription of ASMs was collected using an exploitation form and analyzed using the SPSS version 13 software. Results: Based on questionnaire responses, our results showed that Valproic acid (96.3%) and Lamotrigine (59.8%) were the two most prescribed ASMs for generalized seizure types. For focal seizure types, Carbamazepine (98.8%) and Levetiracetam (34.1%) were the most commonly prescribed drugs, whereas for combined focal and generalized seizure types, the combination of Valproic acid and Carbamazepine (38.55%) was the most prescribed. Phenobarbital was the most commonly prescribed ASM for status epilepticus (40.2%). These prescription preferences were mainly due to seizure types, cost, health insurance coverage, years of experience, and additional epileptology training (p < 0.05). Conclusion: Our results show a shift in the prescription of ASMs in Morocco. Similar to many other countries, valproic acid and carbamazepine are considered the first-line treatments for generalized and focal seizure types. Some factors remain as major challenges in enhancing epilepsy management in Morocco.

Double Cortex Syndrome: An Unusual Cause of Seizures.

Gray matter heterotopia (GMH) is caused by abnormal neuronal migration during brain development. Subcortical band heterotopia (SBH), or double cortex, is a rare variant of GMH that mainly affects female patients with epilepsy (PWE) with different degrees of mental retardation. We present the case of a 25-year-old woman who was admitted to the neurology department of our tertiary hospital with generalized tonic-clonic seizures. Her mother had a normal antenatal period and a history of labor. There was a history of immediate crying and normal appearance, pulse, grimace, activity, and respiration (APGAR) scores. She had delayed milestones, which affected various categories of child development. Physical examination revealed a global developmental delay. Laboratory values, including complete blood count, serum calcium, and arterial blood gas tests, were all within normal limits. An EEG showed significant abnormalities suggestive of epilepsy. An MRI of the brain showed a continuous band of gray matter located deep and parallel to the cortex in both cerebral hemispheres, suggesting double cortex syndrome (DCS).

Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis Presenting With Parkinsonism.

Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT) is a rare condition defined as encephalopathy with a positive antithyroid antibody. We report the case of a 52-year-old woman who presented with Parkinsonism associated with Hashimoto's thyroiditis. A few similar cases have been reported. Our patient responded well to corticosteroids with a significant reduction in symptoms. Diagnosis can pose a significant challenge in SREAT cases because of its variable clinical presentation. Therefore, we recommend evaluating thyroid function and thyroid autoantibodies in the context of acute and subacute encephalopathy. In the elderly population, SREAT, as a cause of Parkinsonism, should not be forgotten because of its simple treatment and significant improvements in neurological symptoms.

Antibiotics-Induced Intracranial Hypertension: A Case Report With Literature Review.

Idiopathic intracranial hypertension (IIH) is a rare condition characterized by increased intracranial pressure, with an unknown cause. However, the pathophysiology of antibiotic-induced IIH remains unclear. The clinical symptoms include headache, visual disturbances, and vomiting. The diagnosis is confirmed by an elevated intracranial pressure (ICP) with normal CSF study and cerebral imaging. Management includes discontinuing the offending antibiotic and reducing ICP with medications such as acetazolamide or diuretics. Therefore, surgical intervention may be necessary in severe cases. In this article, we report the case of a 19-year-old patient, admitted with symptoms of intracranial hypertension syndrome, occurring three days after receiving antibiotics (gentamicin, penicillin). Physical examination revealed bilateral optic disc edema. Cerebral magnetic resonance imaging (MRI) revealed indirect signs of intracranial hypertension. The CSF pressure measurement was approximately 290 mmHg, while CSF and other laboratory blood tests were normal. The patient received methylprednisolone bolus and topiramate (50 mg/day). A month later, the clinical outcome showed regression of headaches and regression of the papilledema.

Behavioral and psychological subsyndromes in Alzheimer's disease using the Neuropsychiatric Inventory.

OBJECTIVE: Behavioral and psychological symptoms of dementia represent common clinical features of dementias, contributing to the heterogeneous phenotypic expression of Alzheimer's disease (AD). During the last two decades, several studies explored the possible presence of neuropsychiatric subsyndromes in dementia by examining the internal structure of the Neuropsychiatric Inventory (NPI). The aim of the present review is to present available evidence coming from studies adopting factor analysis to explore the NPI and describe neuropsychiatric clusters of symptoms in AD. DESIGN: A systematic review of literature was performed concerning available studies describing neuropsychiatric subsyndromes in AD by adopting the NPI. RESULTS: Overall, our analysis showed a relatively low concordance among available evidence for what concerns the definition and composition of NPI clusters, possibly due (at least in part) to the heterogeneity of the sample populations recruited in the studies. However, we also observed some consistent associations of specific symptoms across studies, defining potential subsyndromes in AD. More consistent results were obtained by studies evaluating the 10-item version of the NPI rather than the more recent 12-item one. CONCLUSIONS: This review represents the first attempt to systematically evaluate evidence coming from factor analyses exploring the internal structure of the NPI in order to facilitate the identification of neuropsychiatric syndromes in AD patients. The NPI may support the definition of behavioral subsyndromes in AD. The evaluation of neuropsychiatric subsyndromes should always take into account the main potential confounders, such as age, severity of disease, and concomitant pharmacological treatment.

"The understanding and attitude toward epilepsy in the Souss-Massa region of Morocco".

OBJECTIVE: Sociocultural beliefs about epilepsy impede medical treatment and social acceptance of people with epilepsy. These barriers can lead to disability and contribute to the stigmatization of people with epilepsy. Assessing knowledge, myths, and attitude about epilepsy and associated factors among adults in Souss-Massa, Morocco. METHODS: We conducted a cross-sectional study in which data were collected from a sample of 385 individuals in the Souss-Massa region of Southern Morocco. Data were collected through face-to-face interviews using a structured questionnaire. The analysis of the collected questionnaires was processed using IBM SPSS Statistics 26. RESULTS: 385 responses were included in the analysis. The results reveal a lack of knowledge, an unfavorable attitude, and the persistence of myths and misunderstandings about epilepsy. The findings of this study suggest that good knowledge was significantly associated with sociodemographic factors: the age range of more than 54 years and health professional status. In addition, the favorable attitude toward epilepsy correlates with the sociodemographic factors: male gender and urban residential area. SIGNIFICANCE: The main finding of this study indicates insufficient knowledge, the persistence of myth, and an unfavorable attitude about epilepsy. Therefore, the application of public awareness programs about epilepsy in urban and rural areas is necessary.

Neurocognitive state and quality of life of patients with glioblastoma in Mediterranean countries: a systematic review.

BACKGROUND: Gliomas are the most common primary tumors of the central nervous system (CNS). The most common subtype of glioma in adults is grade IV astrocytoma, known as glioblastoma (GB) multiforme. Despite advances in treatment, GB remains a lethal tumor with a poor prognosis, and patients face serious quality of life (QoL) issues. Its poor prognosis is a real public health problem. The present study aimed to determine the exact neurocognitive status and QoL in patients with GB in the Mediterranean region and the different predictive factors responsible for their deterioration. METHODS: This systematic review was conducted in accordance with PRISMA guidelines. The protocol was registered in the PROSPERO (Identifier: CRD42020188936). The following databases have been independently searched by 2 authors: PubMed, Science Direct, Scopus and Google Scholar. RESULTS: Thirteen studies were selected (n=13). Four studies (n=4) focused solely on cognitive assessment, five studies (n=5) focused on quality-of-life assessment, and four (n=4) were simultaneously assessed QoL and neurocognitive status. The majority of studies in this review use (Mini-Mental State Examination, MMSE) for an overall cognitive assessment (n=5), other studies use specific batteries for an in-depth assessment of cognitive functions (n=3). The study revealed several affected functions: short and long-term memory, executive functions (EFs), and visuo-constructive abilities. Scale of European Organization for Research and Treatment of Cancer and the Functional Assessment of Cancer Therapy are among the most widely used instruments for assessing QoL. For factors influencing neurocognitive status and QoL, the present review found that: The Karnofsky Performance Scale (KPS), tumor location, age, sex and type of treatment are the most identified. Other studies have reported other factors, such as tumor progression, development of emotional distress, and coping strategies adopted. DISCUSSION: We conclude that there were many changes in patients with GB during the course of the disease and that most of them were related to age and disease progression. The use of coping strategies based on social support has a positive impact on the QoL.

Analysis of Fourteen New Cases of Meningovascular Syphilis: Renewed Interest in an Old Problem.

Neurosyphilis (NS) remains a public health problem. Several recent reports suggest a worldwide increase in the incidence of this condition. Various syndromes can occur in NS, such as syphilitic meningitis, meningovascular syphilis, parenchymatous and gummatous neurosyphilis. Syphilis meningovascular will be the focus of this study. We report 14 new observations of meningovascular syphilis. A review of demographic and clinical features, neuroimaging findings, cerebrospinal fluid changes, treatment and outcome, pathophysiology mechanism of meningovascular syphilis are presented.

Misdiagnosed spontaneous intracranial hypotension complicated by subdural hematoma following lumbar puncture.

INTRODUCTION: Spontaneous intracranial hypotension is an infrequent cause of secondary headache due to cerebrospinal fluid (CSF) hypovolemia. OBJECTIVE: To describe a case of headache revealing spontaneous intracranial hypotension complicated by subdural hematoma following lumbar puncture. OBSERVATION: A 34-year-old man presented with acute postural headache. The first cerebral computed tomography scan was normal. Lumbar puncture showed hyperproteinorachy at 2 g/L with six lymphocytic cells. The headache became very intense. At admission, clinical examination was normal. Ophthalmological examination did not show any abnormalities. Encephalic magnetic resonance imaging (MRI) showed bilateral subdural hematoma with tonsillar descent simulating Chiari type I malformation. After surgical drainage and symptomatic treatment, the patient was discharged with no recurrence. CONCLUSION: Spontaneous intracranial hypotension is associated with simple clinical presentation, orthostatic headache, and characteristic MRI findings. Misdiagnosed, it leads to unnecessary procedures.

Effects of Gingko biloba supplementation in Alzheimer's disease patients receiving cholinesterase inhibitors: data from the ICTUS study.

Ginkgo biloba (Gb) is currently the most investigated and adopted herbal remedy for cognitive disorders and Alzheimer's disease (AD). Nevertheless, its efficacy in the prevention and treatment of dementia still remains controversial. Specifically, the added effects of Gb in subjects already receiving "conventional" anti-dementia treatments have been to date very scarcely investigated. We evaluated whether the use of Gb is associated with additional cognitive and functional benefit in AD patients already in treatment with cholinesterase inhibitors (ChEIs). Data are from mild to moderate AD patients under ChEI treatment recruited in the Impact of Cholinergic Treatment USe (ICTUS) study. Mixed model analyses were performed to measure six-monthly modifications in the Mini Mental State Examination (MMSE), the Alzheimer's Disease Assessment Scale-Cognitive (ADAS-Cog) subscale score, and the Activities of Daily Living (ADL) scale over a follow-up of 1 year according to the additional Gb supplementation. A total of 828 subjects were considered for the present analyses. Significantly different modifications at the MMSE score over the 12-month follow-up were reported between patients on combined therapy compared to those only taking ChEIs. On the contrary, the modification of the ADAS-Cog score between the two groups did not show statistically significant differences, although similar trends were noticed. No significant modifications of the two adopted outcome measures were observed at the mid-term 6-month evaluation. The modifications over time of the ADL score did not show statistically significant differences between the two groups of interest. Our findings suggest that Gb may provide some added cognitive benefits in AD patients already under ChEIs treatment. The clinical meaningfulness of such effects remains to be confirmed and clarified.

Impact of behavioral subsyndromes on cognitive decline in Alzheimer's disease: data from the ICTUS study.

Behavioral and psychological symptoms of dementia (BPSD) represent common manifestations among patients affected by Alzheimer's disease (AD). Some reports have recently classified BPSD into specific clusters/subsyndromes exploring the internal structure of the Neuropsychiatric Inventory (NPI). We evaluated whether specific behavioral subsyndromes are associated with worsening cognitive function. Mild to moderate AD patients were recruited from the cohort of the Impact of Cholinergic Treatment USe (ICTUS) study. Neuropsychiatric symptoms were classified in three subsyndromes, identified at baseline, grouping different combinations of NPI items: (1) "psychotic" ("delusions" and/or "hallucinations"); (2) "affective" ("agitation" and/or "depression" and/or "anxiety" and/or "irritability"); and (3) "behavioral" ("euphoria" and/or "apathy" and/or "disinhibition" and/or "aberrant motor behavior"). Mixed model analyses were performed to measure six-monthly changes in the ADAS-Cog score over a follow-up of 2 years, according to these subsyndromes. All analyses were stratified according to AD severity as defined by the Clinical Dementia Rating (CDR). A total of 1,375 AD subjects were recruited. No NPI cluster was found to significantly (p < 0.05) affect the rate of cognitive decline across the 3 CDR classes. Our results suggest that the cognitive course of AD is not substantially influenced by the presence of specific neuropsychiatric phenotypes. Further studies are needed to extend the present findings and identify possible biological and clinical bases for behavioral subsyndromes.

Cognitive interventions targeting subjective cognitive complaints.

Subjective cognitive complaints (SCCs) are being increasingly recognized as a preclinical phase of dementia. Thus, SCCs may represent a "promising" stage for planning and implementing preventive interventions aimed at reducing the incidence of cognitive disorders. The aim of the present study is to present and discuss the available evidence coming from clinical trials adopting cognitive interventions in individuals with SCCs. A systematic review of literature was conducted to evaluate the available trials testing nonpharmacological cognitive interventions for the prevention of dementia in subjects with SCCs. Six studies were included in the present study. Overall, most interventions showed to objectively improve cognitive performance in subjects with SCCs. A relevant heterogeneity was found concerning their characteristics and feasibility. Conversely, there is a current lack of evidence in the literature about the efficacy of nonpharmacological cognitive interventions for preventing dementia or cognitive impairment.