Neurological Manifestations Research Articles

Movement Disorders after Dengue Virus Infection: A Scoping Review.

Movement disorders after dengue virus (DENV) infection have been increasingly recognized. We aimed to synthesize the clinical and paraclinical characteristics, treatment responses, and outcomes of these neurologic complications. We systematically reviewed PubMed, Embase, Scopus, and LILACS databases up to September 2023 following a published protocol. We identified 73 cases of DENV-induced movement disorders. Cerebellar ataxia was the most common, followed by parkinsonism, opsoclonus-myoclonus-ataxia syndrome, and dystonia. Movement disorders typically developed within 14 days of DENV infection and were associated with a range of neurological symptoms, including cognitive impairment and psychiatric disturbances. Neuroimaging studies frequently showed abnormalities in the basal ganglia and brainstem. Treatment varied depending on the specific movement disorder and included corticosteroids, intravenous immunoglobulin, and symptomatic medications. Whereas a handful of cases met the criteria for acute encephalitis, many lacked sufficient data to establish a definitive diagnosis. Para-infectious and postinfectious immune-mediated movement disorders were also reported. A rare case of chronic progressive panencephalitis due to DENV infection highlights the potential for long-term neurological consequences. Other DENV-related complications, such as stroke, pituitary apoplexy, subacute thyroiditis, and metabolic disturbances, can also cause movement disorders. We emphasize the importance of recognizing the diverse neurological manifestations of DENV infection and the need for further research to improve our understanding of the underlying mechanisms and optimize treatment strategies. We propose a more rigorous approach to determining the causality between infection and movement disorder, demanding stronger evidence beyond mere association and advocating for targeted research to fill the existing knowledge gaps. © 2025 International Parkinson and Movement Disorder Society.

Encephalopathy in Sjogren’s Syndrome - A Distinct Neurological Presentation

Sjogren’s syndrome (SS) is an autoimmune disorder characterized by lymphocytic and plasma infiltration of exocrine glands, resulting in dry mouth and kerato-conjunctivitis sicca. The clinical symptoms may range from mucosal dryness to more systematic complaints. Sjogren's syndrome (SS) may be complicated by neurological manifestations.1 We report a 36-year-old woman presenting with quadriparesis, started as lower limb weakness which progressed to the upper limbs, having Guillain-Barre syndrome like pattern. MRI brain showed altered signal intensity in the brainstem, predominantly centered in the pons labelled as autoimmune brainstem encephalitis with distal RTA. With differentials as clippers, autoimmune disease and de-mylinating disease patient underwent an Autoimmune panel which showed Antinuclear Antibody showing speckled SSA/RO pattern and antibodies to SSB/LA were positive suggestive of Sjogren’s syndrome. The diagnosis was confirmed with a minor salivary gland biopsy from the lower lip which was definitive of Sjogren’s syndrome. Patient showed excellent improvement on pulsed steroid therapy. Therefore, this case illustrates a rare, heralding manifestation of Sjogren’s syndrome as brainstem encephalitis with definitely diagnosis based on minor salivary gland biopsy showing complete recovery on early detection and management.

Hypoparathyroidism: diagnosis, management and emerging therapies.

Hypoparathyroidism is characterized by inadequate parathyroid hormone (PTH) secretion or action and results in hypocalcaemia, and canlead tohyperphosphataemia and hypercalciuria. Most cases of hypoparathyroidism occur as a complication of surgery, with the remainder due to causes including autoimmune disease, genetic causes, infiltrative diseases, mineral deposition or due to abnormalities in serum levels of magnesium. Hypoparathyroidism can cause multisystem disease, with long-term complications resulting from ectopic calcification as well as renal complications with nephrocalcinosis, nephrolithiasis and renal impairment in addition to respiratory, cardiac or neurological manifestations. Conventional therapy consists of oral calcium salts and active vitamin D but it has limitations, including fluctuations in serum levels of calciumand a high pill burden, and can increase therisk of long-term complications. By contrast, PTH replacement therapy can effectively achieve normal serum levels of calcium, and lower serum levels of phosphate.The long-acting PTH analogue, palopegteriparatide, has been shown to normalize urine levels of calcium. In addition, PTH replacement therapy reduces the pill burden. Palopegteriparatide is alsoassociated with improved quality of life in comparison toconventional therapy. This Review summarizes currentrecommendations regarding the pathophysiology, evaluation and management of hypoparathyroidism and also referencesthe 2022 international hypoparathyroidism guidelines. Palopegteriparatide hasnow been approved as PTH replacement therapy for hypoparathyroidism. Emerging therapies will also be presented in this Review.

Neurological manifestations in patients with VEXAS syndrome.

VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently described syndrome linked to somatic mutations in the UBA1 gene, causing systemic autoinflammatory manifestations. To date, few data are available concerning neurological manifestations. The aim of this study was to describe their prevalence, clinical spectrum and outcome under treatment. Retrospective multicentre study including patients with VEXAS syndrome from the French VEXAS Registry between November 2020 and March 2023. Additional cases were included after a national call for observations. Each patient with confirmed UBA1 somatic mutation and neurological manifestation was reviewed during multidisciplinary meetings. Clinical, radiological, biological characteristics, treatments, and outcome were described. Of the 291 patients included in the French VEXAS Registry, 17 (6%) had central (CNS) or peripheral (PNS) neurological involvement, with 13 additional cases identified by the national call. Of the 30 patients included, 21 (70%) had PNS involvement and 9 (30%) CNS involvement. PNS involvements included polyneuropathy (n = 9), cranial nerve involvement (n = 7), non-length-dependent polyneuropathy (n = 5) and multiple mononeuropathy (n = 3). CNS involvements included encephalopathy (n = 6), lacunar cerebral infarcts (n = 4), posterior reversible encephalopathy syndrome (n = 3) and optic perineuritis (n = 2). Most neurological manifestations were improved by steroids (68%), steroid-sparing agents were used in 90% [most frequently ruxolitinib (n = 11), azacitidine (n = 8), tocilizumab (n = 4)], and mortality was 30% after a median follow-up of 4 years. Neurological manifestations may occur in a small but possibly underestimated proportion of patients with VEXAS syndrome, are heterogeneous and can involve both PNS and CNS.

Updates on neurologic manifestations of mitochondrial disease.

Primary mitochondrial disease (PMD) is diverse both genetically and phenotypically. Neurologic manifestations are present at a high rate and often pose complications for providers. The review will discuss common manifestations and how advances in genetic testing have broadened understanding of PMDs. Across all areas of PMD research, genetic advancements are notable both for mitochondrial and nuclear DNA. Global understanding of PMDs is driving deeper and broader research. Neurologic manifestations primarily include neuromuscular disease, epilepsy, stroke-like episodes and neurodegeneration, and advances in all areas have benefitted from global reporting of genetic studies.

Implications of the choroid plexus in Niemann-Pick disease Type C neuropathogenesis.

Niemann-Pick Disease Type C (NPC) is an ultra-rare disorder characterized by progressive psychiatric and neurologic manifestations, with late infantile, juvenile, and adolescent/adult presentations. We examined morphological properties of the choroid plexus, a protective blood-cerebrospinal fluid barrier, in NPC, and their relationship with neurodegeneration, clinical status, and circulatory markers. This study also determined whether choroid plexus morphology differentiates between NPC and more prevalent illnesses, schizophrenia (SZ) and bipolar disorder (BD), which have overlapping psychiatric symptoms with adolescent and adult-onset NPC and are associated with misdiagnosis. Patients with NPC were assessed using neuroimaging, clinical instruments, and plasma protein quantification focusing on inflammatory markers. Morphological properties (i.e., choroid plexus volumes) were compared between patients with NPC (n=17), SZ (n=20), BD (n=24), and healthy controls (HCs, n=106). Choroid plexus enlargement (p<0.05) and reduced thalamic volumes (p<0.05) were observed in NPC patients versus HCs and SZ or BD patients. A logistic regression model with choroid plexus and thalamic volumes as predictors yielded high prediction accuracy for NPC vs. HCs, NPC vs. SZ, and NPC vs. BD (area under the receiver operating characteristics curve [AUROC] of 1). Choroid plexus volumes were negatively correlated with left (p=0.009-0.012) and right (p=0.007-0.025) thalamic volumes, left (r=-0.69, p=0.003) and right (r=-0.71, p=0.002) crus I of the cerebellum, and greater severity on the NPC-Suspicion Index psychiatric subscale (ρ=0.72, p=0.042). Targeted protein expression quantification revealed differential expression of TGFA, HLA-DRA, TNFSF12, EGF, INFG, and IL-18 in NPC patients vs. HCs (p<0.05), with higher choroid plexus volumes correlating with IL-18 levels (ρ=0.71, p=0.047). The choroid plexus may play a critical role in NPC neuropathogenesis and serve as a novel biomarker for monitoring neurodegenerative and inflammatory processes in NPC.

Assessing cognitive functions in non-neuropsychiatric childhood systemic lupus erythematosus: Cross-sectional study.

Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by multisystem, including neuropsychiatric, involvement. The nervous system is affected in 20-27% of patients within approximately two years after diagnosis. This study aimed to examine neurocognitive impairment in childhood-onset SLE (cSLE) patients before the development of any neurological, psychiatric, or cognitive manifestations. The study included 98 children and adolescents. Of these, 34 (35%) were cSLE groups, and the remaining formed two control groups: 31 (31%) oligoarticular Juvenile Idiopathic Arthritis (oJIA) patients served as a "patient control" group, and 33 (34%) healthy participants socio-demographically matched to the cSLE and oJIA patients formed the healthy control (HC) group. None of the subjects in the study exhibited neurological or psychiatric symptoms. The Wechsler Intelligence Scale for Children-IV (WISC-IV) was applied to all groups. Test results showed that the Perceptual Reasoning Index Score (PRIS) was significantly lower in cSLE than in HC (F(2, 95)=3.365, p=0.042, Tukey HSD: p=0.01). 'Comprehension' subtest scores were significantly lower in the cSLE group compared to the HC group (H(2)=8.133, p=0.017; U=352.5, p=0.009). Similarly, 'symbol search' subtest scores were significantly lower in the cSLE group compared to the HC group (F(2, 95)=3.545, p=0.036, Tukey HSD: p=0.014). Our results revealed that cSLE may have neurocognitive impairment without presenting any symptoms. Early detection is possible with the neurocognitive test WISC-IV. These results support the inclusion of objective neurocognitive assessment methods into the routine clinical follow-up of childhood-onset SLE.

Should antinuclear antibodies (ANA) be used to screen for connective tissue disease in neurological patients?

Patients with connective tissue diseases (CTD) can have a wide range of neurological manifestations. Neurological complaints may be the presenting symptom of CTD. Therefore, screening for CTD using anti-nuclear antibodies (ANA) is a common practice. However, due to the abundance of positive ANA in a healthy population, interpretation of the results may be complex. we retrospectively evaluated files of patients hospitalized for evaluation of neurological symptoms in Sheba Medical Center during the years 2007-2022. Data was collected regarding epidemiology, ANA status, and rheumatological diagnosis. 4723 patients' files were reviewed. Of them, 46.6% were positive for ANA. 6.9% of them were diagnosed with CTD. This population had significantly higher rates of positive ANA status (71.2% vs 28.8%, p<0.001), was significantly older (59.4 vs 53.4years, p<0.001) and had a significantly higher ANA titer (1:484.8, 1:268 p<0.001) compared to patients without CTD. Factors which were found predictive for CTD diagnosis included female gender, older age, ANA titer above 1:160, and the diagnosis of a non-vascular etiology for the neurological disease. Females, older patients, patients with high ANA titer and with diagnosis of a non-vascular cause to their neurological complains may be more likely to harbor a CTD and should probably be further evaluated.

Complex orbitocranial penetrating injury-managed through neuroplastic surgery – a rare case report and review of literature

Introduction and importance: Orbitocranial penetrating injuries (OPI) are uncommon types of head injuries, accounting for about 0.04% of all head injuries. Foreign bodies penetrating the orbit and cranium cause fractures, leading to ophthalmic and neurological manifestations. These require a specialized treatment approach that mainly depends on the foreign body type, extent of injury, and potential complications. Management involves foreign body removal, reconstruction, and infection control. Case details: We discuss a case of a 25-year-old male who presented with a penetrating cranio-orbital injury caused by a wooden stick through the left eye. The transorbital-transcranial wooden foreign body penetrated the orbital roof and temporal bone causing displacement of the orbital contents and extended intracranially into the temporal region. The management was done by a multidisciplinary approach consisting of a team of neurosurgeons and oculoplastic surgeons by extracting the foreign body and reconstruction. Clinical discussion: No sound literature on the management of OPI by neuroplastic surgery is available. Conclusion: This article highlights the importance of neuroplastic surgery for reconstructing complex fractures involving the cranio-orbital and craniofacial regions for improving patient outcomes, and preventing potential complications. This could potentially revolutionize the management of complex craniofacial abnormalities and a new era for neuro-reconstructive care.

Cutaneous Manifestations of NAXD or NAXE Deficiency: A Literature Review for the Dermatologist.

Nicotinamide adenine dinucleotide phosphate hydrate dehydratase (NAXD) and nicotinamide adenine dinucleotide phosphate hydrate epimerase (NAXE) deficiencies are rare autosomal recessive metabolic disorders characterized by severe neurological manifestations during infancy. In affected individuals, febrile illnesses can trigger progressive encephalopathy often accompanied by distinctive skin eruptions, resulting in high mortality rates. This study reviews the literature on NAXD/NAXE deficiencies, focusing on dermatological manifestations and their correlation with genotypic variations and treatment responses. A comprehensive literature search identified 45 patients with 31 pathogenic/likely pathogenic mutations, and a median age of onset at 1.16 years. Patients with NAXE deficiency exhibited a broader range of age of symptom onset compared to those affected with NAXD deficiency. Fever or infections were identified as the most common triggers for decompensation episodes. Skin manifestations were observed in 31% of patients with whole cell NAXD and NAXE deficiencies. The characteristic skin eruption comprises well-demarcated erythematous and erosive plaques progressing to blistering and necrosis, predominantly affecting flexural surfaces. The mortality rate was 78%, with survivors experiencing varying degrees of neurological sequelae. Niacin/nicotinamide supplementation resulted in improvements in skin lesions and survival rates. The review underscores the critical role of dermatologists in early diagnosis and intervention of NAXE and NAXD deficiencies. Recognizing characteristic skin manifestations is essential, particularly in patients with acute neurological decline following infections or vaccinations. Early intervention with specific supplements shows promise; however, further research is needed to establish standardized treatment protocols and enhance patient outcomes.

Tuberculosis menace: a case report of disseminated tuberculosis presenting as tubercular meningitis and long-segment cervical tuberculous myelitis in a 32-year-old man from rural India

This case report discusses an uncommon presentation of miliary tuberculosis as tubercular meningitis (TBM) and long-segment cervical tuberculous myelitis in a 32-year-old man from rural India. The patient presented with symptoms of fever, headache, neck stiffness, and gradual weakness in all four limbs. Hydrocephalic changes secondary to meningitis and involvement of the spinal cord were observed on neuroimaging and were correlated with clinical findings of cervical myelitis, confirming the diagnosis of TBM with cervical myelitis. TBM, together with cervical myelitis in patients with miliary tuberculosis (TB), is a rare manifestation in tubercular endemic countries, such as India. It is crucial to confirm the diagnosis and initiate antitubercular therapy (ATT) promptly in order to prevent neurological complications. In this context, the present case highlights the importance of considering TB in patients with neurological manifestations that are not characteristic of the most common diseases. This report also emphasizes the need to raise awareness and improve the management of tuberculosis in rural areas where there are few opportunities to access tertiary centers.

Neurological Manifestations Associated With Hypomagnesemia Due to Proton-Pump Inhibitors: A Case Series

Neurological Manifestations Associated With Hypomagnesemia Due to Proton-Pump Inhibitors: A Case Series

P-678. Comparison between the Characteristics and Outcomes of Patients Hospitalized for COVID-19 in Three Waves of the Pandemic

Abstract Background Throughout the COVID-19 pandemic, new SARS-CoV-2 variants emerged, each prevailing during a certain time period and defining the pandemic waves that were observed all over the world. Even in different countries, such waves presented many similarities, such as the period of occurrence, clinical characteristics of those who were infected by the SARS-CoV-2 virus, COVID-19 outcomes and governmental public policies to stop the spread of the disease. Their major differences are believed to be related to the SARS-CoV-2 variant circulating at the time, as well as the availability of vaccines, patient vulnerabilities, resource availability and knowledge in patient management. Methods This study is part of a multicentric retrospective cohort, entitled the Brazilian COVID-19 Registry, which included adult patients (≥18 years), with laboratory-confirmed COVID-19, admitted between March 2020 and August 2022 in 41 hospitals in six Brazilian states. The primary outcome was in-hospital mortality. Secondary outcomes included: intensive care unit (ICU) admission, need for mechanical ventilation, kidney replacement therapy (KRT) thromboembolic event, nosocomial infection, acute heart failure and septic shock. Results The median age decreased in the second wave and increased in the third compared to the first. The total number of comorbidities was higher during the third wave and lower in the second. When evaluating symptoms, in the third wave, anosmia, ageusia and fever were less common when compared to the other waves , while neurological manifestations were more frequent. The frequency of admission to the intensive care unit and in-hospital mortality reduced throughout the pandemic. Conclusion The study showed that clinical manifestations and outcomes changed between the three COVID-19 waves. In the second phase of the pandemic, patients hospitalized with Covid-19 were younger and had fewer comorbidities. The risk of severe disease has decreased substantially since the start of the pandemic. However, the new strains are under continued investigation, because of the unknown impacts in severity and vaccine effectiveness, the response to existing therapies and the accuracy of diagnostic tools. Disclosures All Authors: No reported disclosures

MR Imaging in Dengue Patients Presenting with Neurological and Ocular Manifestation: A Case Series in Tertiary Care Hospital

MR Imaging in Dengue Patients Presenting with Neurological and Ocular Manifestation: A Case Series in Tertiary Care Hospital

The probable progression of Epstein-Barr virus (EBV) to chronic active EBV/reactivation weakens the immune response and stimulates Cryptococcus neoformans infection, which invariably proves fatal: a case report and review of the literature

We have reported here the fatal outcome of Epstein-Barr virus (EBV) infection in a 58-year-old male who had probably developed reactivation/chronic active EBV (CAEBV) which gave rise to various neurological deficits, pancytopenia, and a lower CD4 count in the patient. The decreased immune response helped Cryptococcus neoformans (C. neoformans) to manifest a disseminated infection. Although he was exclusively provided with antifungal treatment and the patient appeared to be successfully treated for cryptococcal infection, no coverage of EBV appeared detrimental as the patient died the very next day. This report highlights the need for clinical suspicion of EBV in unexplained cases of neurological manifestation, the hematological disorder of pancytopenia, a lower CD4 count, and multiorgan involvement such as pleural effusion, coarse liver echotexture, and splenomegaly.

The neurotoxicity of iodoacetic acid, a byproduct of drinking water disinfection

IAA is a by-product of the water disinfection process and has been found to be neurotoxic. However, the role and mechanism of IAA neurotoxicity remain unclear. In this review, we comprehensively discuss the neurotoxic effects and mechanisms of IAA from the molecular level, cellular level and neurological manifestations. At the molecular level, IAA causes neurotoxicity by reducing mitochondrial membrane potential, aggravating oxidative stress and DNA damage. At the cellular level, IAA causes neurotoxicity by inducing BBB disruption, neuroinflammation, and apoptosis. In neurological manifestations, IAA can lead to neurotransmitter disorders, neurodevelopment dysfunction, and even neurodegenerative diseases. Taken together, our review provides insights into the mechanisms of IAA neurotoxicity that will contribute to future studies of IAA neurotoxicity and its protective strategies.

ASSESSMENT OF BODY COMPOSITIONS BY FOOT-TO-FOOT BIOELECTRIC IMPEDANCE AMONG CHILDREN WITH NEUROLOGICAL MANIFESTATIONS

OBJECTIVE: In clinical practice, the use of foot-foot bioelectrical impedance analysis (BIA) in the assessment of body composition continues to increase. Its preference is primarily defined by its portability, simplicity, speed, and reliability. The present study aimed to evaluate body composition by BIA among children with neurological manifestations. MATERIAL AND METHODS: Anthropometric measurements and body composition of 406 children attending the pediatric neurology outpatient clinic were analyzed, and the gender and groups were compared based on the diagnosis. RESULTS: Boys manifested lower values of weight, percentage body fat (%BF), fat percentile, fat mass, and Body mass index (BMI) but higher values of percentage total body water (%TBW) than girls [weight (p = 0.015), %BF (p&amp;lt;0.001), fat percentile (p = 0.001), fat mass (p&amp;lt;0.001), BMI (p = 0.006), and %TBW (p&amp;lt;0.001)]. Significant differences were determined among the groups except for weight standard deviation score (SDS) and height SDS (p&amp;lt;0.001). CONCLUSIONS: Based on body composition evaluation by foot-to-foot BIA, We may suggest that each neurological subgroup within the pediatric population demonstrates a distinct body composition. Therefore, evaluation of body composition will contribute to obtaining information about nutritional/hydration status..

Heterozygous variants in AP4S1 are not associated with a neurological phenotype.

Biallelic loss-of-function variants in AP4S1 cause childhood-onset hereditary spastic paraplegia. A recent report suggested that heterozygous AP4S1 variants lead to a syndrome of lower limb spasticity and dysregulation of sphincter function. We critically evaluate this claim against clinical observations in 28 heterozygous carriers of the same AP4S1 variant (NM_007077.3: c.289C>T, p.Arg97Ter). In these 14 males and 14 females (mean age: 37.6 ± 4.9 years [SD], range: 30-50 years), we ascertain no increased prevalence of neurological manifestations. Alternative causes should be considered when evaluating patients with heterozygous AP4S1 variants and neurological symptoms, as misattribution of pathogenicity can impact clinical care and genetic counseling.

TREATMENT RESISTANT EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME) WITH NEUROLOGICAL COMPLICATIONS: A CASE REPORT

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a rare autoimmune vasculitis affecting small- and medium-sized blood vessels. It is characterized by multisystem involvement, including respiratory, dermatological, and neurological manifestations. Neurological complications, such as mononeuritis multiplex and peripheral neuropathy, are reported in up to 70% of cases and can result in significant functional impairment. Standard treatments, including glucocorticoids and cyclophosphamide, may fail in refractory cases, necessitating alternative therapeutic approaches. Case: A 23-year-old female with a known history of asthma presented with progressive shortness of breath, generalized weakness, and neurological symptoms, including seizures, bilateral leg rashes, and limb paralysis, leading to bed confinement. Initial misdiagnosis and antituberculous therapy delayed appropriate management. Imaging studies, clinical findings, and laboratory investigations confirmed a diagnosis of EGPA with significant peripheral neuropathy and mononeuritis multiplex. Despite six cycles of cyclophosphamide and glucocorticoids, the patient exhibited no improvement, indicating treatment resistance. Monoclonal antibody therapy with rituximab was initiated, resulting in marked improvement in both respiratory and neurological symptoms. The patient remains under regular follow-up, with sustained symptomatic relief and improved functional status. Conclusion: This case highlights the challenges in diagnosing and managing refractory EGPA, particularly in young patients with atypical presentations and severe neurological complications. The successful response to rituximab underscores its potential as an effective therapeutic option in treatment-resistant cases. It further emphasizes the importance of early diagnosis, multidisciplinary care, and the need for personalized treatment strategies to optimize outcomes for EGPA patients.

Neuropsychiatric manifestations in systemic lupus erythematosus and Sjogren's disease.

Autoimmune diseases often present in a systemic manner, affecting various organs and tissues. Involvement of the central and peripheral nervous system is not uncommon in these conditions and is associated with high morbidity and mortality. Therefore, early recognition of the neuropsychiatric manifestations associated with rheumatologic diseases is essential for the introduction of appropriate therapies with the objective of providing a better quality of life for individuals. To provide a literature review of the neuropsychiatric manifestations related to Systemic Lupus Erythematosus (SLE) and primary Sjögren's Disease (pSD), through the description of signs, symptoms, and immunological variables associated with these conditions. A literature review was conducted by searching for national and international articles available in the SciELO and PubMed databases related to the description of neurological and psychiatric manifestations in patients with the rheumatologic diseases of interest in this study. The main NP manifestations presented in SLE and pSD are discussed, focusing on clinical presentation and etiology. Treatment option are, however, mainly based on expert opinion, since a few randomized controlled trials have been done. There is a high prevalence of neuropsychiatric manifestations associated with SLE and pSD. The variety of physiopathology pathways may explain the variety of symptoms, however pathological findings are rare. Multicenter studies on attribution protocols and treatment are necessary to address the current gaps.