Neurophysiological Findings Research Articles

Chronic Immune Sensory Polyradiculopathy (CISP): A Systematic Review of the Literature

Chronic immune sensory polyradiculopathy (CISP) is a rare inflammatory immune disorder affecting the nervous system, primarily targeting the proximal sensory nerve roots. The condition was first described by Sinreich in 2004. We conducted a systematic review of CISP cases published on PubMed to identify common clinical presentations, along with neurophysiological, radiological, cerebrospinal fluid (CSF), and other findings. Our review included a total of 22 patients from 8 articles. Many patients presented with gait difficulties and sensory ataxia and were found to have normal nerve conduction studies (NCS) and electromyography (EMG) but exhibited characteristic abnormalities in somatosensory evoked potentials (SSEP), elevated CSF protein levels, thickened nerve roots on contrast-enhanced lumbar spine MRIs, and histological changes on nerve root biopsies. Clinical improvement was observed following treatment with steroids and/or intravenous immunoglobulin (IVIG). The study concluded that while CISP is rare, it is an important clinical entity to consider, as accurate diagnosis and appropriate treatment can lead to significant improvements in neurological symptoms and disabilities.

Phrenic Nerve Sonography Alterations in Patients with ALS: Insight with Clinical and Neurophysiological Findings

Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder, and although the diagnosis is primarily based on clinical criteria, ENMG, as the “gold standard”, does not always show detectable changes. Therefore, our study suggests that alterations in echogenicity and heterogeneity of the phrenic nerve (PN) may serve as potential additional diagnostic tools for ALS. Methods: Our study included 32 patients in the ALS group and 64 individuals in the control group. Each participant underwent an interview and completed questionnaires to collect clinical and demographic data, including age, gender, height, body mass index (BMI), hip and waist circumference, duration of illness, ALS-FRS-R score, comorbidities, and medication use. Ultrasound examinations of the PN were performed by two authors using a high-resolution “Philips EPIQ 7” ultrasound machine equipped with a linear 4–18 MHz transducer. The ALS group participants underwent PN sonography and conduction examinations, arterial blood gas (ABG) analysis, respiratory function tests (RFT), and electroneuromyography (ENMG). Results: The study demonstrated that the phrenic nerve is significantly smaller on both sides in patients with ALS compared to the control group (p < 0.01). Changes in the homogeneity and echogenicity of the PN were also observed on both sides. On the right side, 43.8% of the nerves showed heterogeneity, 40.6% were isoechoic, and 21.9% were hyperechoic. On the left side, 59.4% of the nerves exhibited heterogeneity, 34.4% were isoechoic, and 28.1% were hyperechoic. Moreover, sonography on both sides showed significant correlation with ALS-FRS-R, COMPASS-31, and ENMG results. Conclusions: Our study highlights the importance of phrenic nerve ultrasound as a promising supplementary diagnostic tool for ALS. The significant differences in phrenic nerve size, echogenicity, and homogeneity between patients with ALS and the control group demonstrate that ultrasound imaging can detect morphological changes in the phrenic nerve. Incorporating phrenic nerve ultrasound into routine diagnostic protocols could improve early detection, enhance disease monitoring, and offer a more comprehensive understanding of the neurodegenerative processes in ALS.

CHRNE-related Congenital Myasthenic Syndrome in Iran: Clinical and Molecular Insights

Variants in the CHRNE gene can lead to a condition called congenital myasthenic syndrome (CMS), which affects the neuromuscular junction (NMJ). CHRNE mutations are the most common cause of CMS. Seventy-seven patients with a possible diagnosis of CMS were referred to the neuromuscular clinic of Shariati Hospital affiliated with the Tehran University of Medical Sciences. We performed whole-exome sequencing (WES) to determine the underlying defect in a group of individuals with a possible diagnosis of CMS. Clinical features and morphological and molecular data on 33 patients with mutations in CHRNE were described. Age of onset, age at diagnosis, consanguinity, family history, motor milestone delay, ophthalmoparesis, generalized fatigue, dysphagia, neurophysiologic findings, and response to treatment of the patients were assessed. Nineteen CHRNE variants including 10 novel ones were identified. The most common mutations were c.1327del; (p.Glu443LysfsTer64) in four different families and c.1252-1267dup; (p.Cys423SerfsTer38) in three families. Clinical onset was mostly at birth or under one year with bilateral fatigable ptosis, ophthalmoplegia, bulbar weakness, and proximal muscle weakness. All patients were treated with pyridostigmine ± salbutamol, which resulted in improvement of motor function, dysphagia, and breathing.

Feature-specific divisive normalization improves natural image encoding for depth perception.

Vision science and visual neuroscience seek to understand how stimulus and sensor properties limit the precision with which behaviorally-relevant latent variables are encoded and decoded. In the primate visual system, binocular disparity-the canonical cue for stereo-depth perception-is initially encoded by a set of binocular receptive fields with a range of spatial frequency preferences. Here, with a stereo-image database having ground-truth disparity information at each pixel, we examine how response normalization and receptive field properties determine the fidelity with which binocular disparity is encoded in natural scenes. We quantify encoding fidelity by computing the Fisher information carried by the normalized receptive field responses. Several findings emerge from an analysis of the response statistics. First, broadband (or feature-unspecific) normalization yields Laplace-distributed receptive field responses, and narrowband (or feature-specific) normalization yields Gaussian-distributed receptive field responses. Second, the Fisher information in narrowband-normalized responses is larger than in broadband-normalized responses by a scale factor that grows with population size. Third, the most useful spatial frequency decreases with stimulus size and the range of spatial frequencies that is useful for encoding a given disparity decreases with disparity magnitude, consistent with neurophysiological findings. Fourth, the predicted patterns of psychophysical performance, and absolute detection threshold, match human performance with natural and artificial stimuli. The current computational efforts establish a new functional role for response normalization, and bring us closer to understanding the principles that should govern the design of neural systems that support perception in natural scenes.

Clinical and genetic features of CMT2T in Italian patients confirm the importance of MME pathogenic variants in idiopathic, late‐onset axonal neuropathies

AbstractBackground and AimsSince 2016, biallelic mutations in the membrane metalloendopeptidase (MME) gene have been associated with late‐onset recessive CMT2 (CMT2T). More recently, heterozygous mutations have also been identified in familial and sporadic patients with late‐onset axonal neuropathy, ranging from subclinical to severe. This indicates that the heterozygous MME variants may not be fully penetrant, or alternatively, that they may be a potential risk factor for neuropathy. Here, we describe the clinical, neurophysiological, and genetic findings of 32 CM2T Italian patients.MethodsThe patients were recruited from four different Italian referral centers. Following a comprehensive battery of neurological, electrophysiological, and laboratory examinations, the patients' DNA was subjected to sequencing in order to identify any variants in the gene. Bioinformatic and modeling analyses were performed to evaluate the identified variants' effects.ResultsWe observe a relatively mild axonal sensory‐motor neuropathy with a greater impairment of the lower extremities. Biallelic and monoallelic patients exhibit comparable disease severity, with an earlier onset observed in those with biallelic variants. When considering a subgroup with more than 10 years of disease, it becomes evident that biallelic patients exhibit a more severe form of neuropathy. This suggests that they are more prone to quick progression.InterpretationCM2T has been definitively defined as a late‐onset neuropathy, with a typical onset in the fifth to sixth decades of life and a more rapidly progressing worsening for biallelic patients. CMT2T can be included in the neuropathies of the elderly, particularly if MME variants heterozygous patients are included.

Focal myositis: a literature review of clinical and immunopathological aspects.

Focal myositis (FM) is a rare and restricted skeletal muscle inflammation, presenting as a solid mass with a typical lower leg localization and benign prognosis. In most cases the process solves spontaneously or after immunosuppressant therapy, but sometimes it recurs or progresses to a systemic inflammation. The basis of the disease are mostly unknown. Hence, we provide an update of histopathological features of FM, in order to better define the underlying pathomechanisms of this disorder. A PubMed literature search was focused on the case reports published in English from July 1977 to December 2023. FM and other myositis may show similar morphological features. Emerging studies on MMP molecules and future eventual research on microRNAs (miRNAs) could help in differential diagnosis. Clinical, laboratory, neurophysiological and imaging findings can allow a correct diagnosis. However, muscle biopsy seems to be the only diagnostic tool to differentiate among FM and other localized soft tissue masses.

Threatening an Illusory Limb: An Event-related Potential Study of the Rubber Hand Illusion.

The rubber hand illusion (RHI) is a well-established method for studying body ownership: Given adequate concordance of visual, sensory, and proprioceptive stimuli, the individual experiences a rubber hand as his or her own. To study the effects of a threat to the rubber hand. We created a typical RHI paradigm but added threatening pain: Both the real hand of an individual and the rubber hand were stroked with a brush, either synchronously (RHI-inducing condition) or asynchronously (control condition), but only the rubber hand was then pricked with a needle to create a threatening pain condition. Event-related potentials (ERPs) were recorded in a group of 23 typically-developed individuals. ERP effects were source-localized using low-resolution electromagnetic tomography. The individuals consistently reported experience of the RHI during the experiment when the brush strokes were applied synchronously to both the real hand and the rubber hand. ERP analysis revealed that synchronous brush stroking gave rise to higher amplitude of frontal ERPs in the 100-200 ms range than asynchronous brush stroking, which was interpreted as reflecting the RHI. In the threatening pain condition, ERPs showed a greater positivity at frontocentral electrodes, source localized in the supplementary motor area (SMA). SMA activation could reflect a control mechanism over reflexive motor activity, facilitating a possible threat-related response. Further studies should address ERP effects and the extent of the RHI to standard and threat stimuli in a correlative fashion to further elucidate the functional significance of the neurophysiological findings.

Stroke and Guillain-Barre syndrome secondary to spotted fever –A case report

Rickettsial infection is an important differential diagnosis in prolonged febrile illness and in returned travellers.We report a 44-year-old male from Deraniyagala, Sri Lanka, with spotted fever Rickettsiosis presenting with stroke and Guillain-Barre syndrome. He presented with dysarthria, reduced sensorium, and acute renal failure on the background of a prolonged febrile illness over two weeks. Following admission there was rapid deterioration of consciousness due to renal failure requiring intubation and repeated haemodialysis. On brain imaging, there were bilateral basal ganglia and internal and external capsule infarctions. His serology was positive for spotted fever Rickettsial infection and there was rapid improvement following treatment with doxycycline. However, difficulty in weaning off from the ventilator due to poor respiratory effort and symmetrical flaccid quadriparesis with areflexia raised concerns of concomitant Guillain-Barre syndrome. Serial cerebrospinal fluid (CSF) studies showed the development of elevated CSF protein with cytoalbuminologic dissociation during his stay in the intensive care unit (ICU). A diagnosis of co-existing Guillain-Barre syndrome was supported by the neuro-physiological findings and marked motor improvement following therapeutic plasmapheresis. This unmasked the right-sided mild residual hemiparesis he suffered due to stroke.

Dropping Objects in Carpal Tunnel Syndrome: Clinical and Electrophysiological Features.

Background Dysfunction in both afferent sensory and efferent motor components of the median nerve may contribute to the manifestation of the symptom of dropping objects (DO) in carpal tunnel syndrome (CTS). The objective of this study was to compare the clinical and electrophysiological characteristics of CTS patients with dropping objects (wDO) and those without dropping objects (w/oDO). The study evaluated the correlation between DO symptoms and median partial conduction block, as well as the reduction in median motor conduction velocity at the wrist. Methodology An examination for CTSand the Boston Carpal Tunnel Syndrome Questionnaire (BCTQ) were performed. Median and ulnar sensory and motor nerve conduction studies were conducted in both upper extremities, and the percentage decrease in the median compound motor action potential amplitude at the wrist level was calculated. Results The study included 201 female participants aged between 22 and 88 years, comprising 57 patients withCTSwDO, 78 patients with CTSw/oDO, and 66 healthy controls. In the CTSwDO group, compared to the CTSw/oDO group, BCTQscores were significantly higher, the prevalence of sensory deficits was more pronounced, and a larger number of hands, both dominant and non-dominant, were classified as stage 3 or higher in the clinical CTS classification. However, no correlation was found between the presence of DO symptoms and any neurophysiological findings. Conclusions DO may be a symptom of clinical severity in CTS, as previously reported.No neurophysiological parameter that could be associated with DO was identified.

A phase 2, open-label study of anti-inflammatory NE3107 in patients with dementias.

Alzheimer's disease (AD) is a progressive, multifactorial, neurodegenerative disorder affecting >6 million Americans. Chronic, low-grade neuroinflammation, and insulin resistance may drive AD pathogenesis. We explored the neurophysiological and neuropsychological effects of NE3107, an oral, anti-inflammatory, insulin-sensitizing molecule, in AD. In this phase 2, open-label study, 23 patients with mild cognitive impairment or mild dementia received 20-mg oral NE3107 twice daily for 3 months. Primary endpoints assessed changes from baseline in neurophysiological health and oxidative stress (glutathione level) using advanced neuroimaging analyses. Secondary endpoints evaluated changes from baseline in neuropsychological health using cognitive assessments, including the 11-item Alzheimer's Disease Assessment Scale-Cognitive Subscale (ADAS-Cog11), Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment, Clinical Dementia Rating, Quick Dementia Rating Scale, Alzheimer's Disease Composite Score, and Global Rating of Change (GRC). Exploratory endpoints assessed changes from baseline in neuroinflammation biomarkers (tumor necrosis factor alpha, TNF-α) and AD (amyloid beta and phosphorylated tau [P-tau]). NE3107 was associated with clinician-rated improvements in cerebral blood flow and functional connectivity within the brain. In patients with MMSE ≥ 20 (mild cognitive impairment to mild AD; n = 17), NE3107 was associated with directional, but statistically nonsignificant, changes in brain glutathione levels, along with statistically significant improvements in ADAS-Cog11 (P = .017), Clinical Dementia Rating (P = .042), Quick Dementia Rating Scale (P = .002), Alzheimer's Disease Composite Score (P = .0094), and clinician-rated GRC (P < .001), as well as in cerebrospinal fluid P-tau levels (P = .034) and P-tau:amyloid beta 42 ratio (P = .04). Biomarker analyses also demonstrated directional, but statistically non-significant, changes in plasma TNF-α, consistent with the expected mechanism of NE3107. Importantly, we observed a statistically significant correlation (r = 0.59) between improvements in TNF-α levels and ADAS-Cog11 scores (P = .026) in patients with baseline MMSE ≥ 20. Our results indicate that in this study NE3107 was associated with what appear to be positive neurophysiological and neuropsychological findings, as well as evidence of improvement in biomarkers associated with neuroinflammation and AD in patients diagnosed with dementia. Our findings are consistent with previous preclinical and clinical observations and highlight a central role of neuroinflammation in AD pathogenesis.

An exploratory study of the damage markers NfL, GFAP, and t-Tau, in cerebrospinal fluid and other findings from a patient cohort enriched for suspected autoimmune psychiatric disease

There is growing evidence suggesting that immunological mechanisms play a significant role in the development of psychiatric symptoms in certain patient subgroups. However, the relationship between clinical red flags for suspected autoimmune psychiatric disease and signs of central nervous system (CNS) pathology (e.g., routine cerebrospinal fluid (CSF) alterations, CNS damage markers, neurophysiological or neuroimaging findings) has received limited attention. Here, we aimed to describe the prevalence and distribution of potential CNS pathologies in psychiatric patients in relation to clinical red flags for autoimmune psychiatric disease and psychiatric symptoms. CSF routine findings and CNS damage markers; neurofilament light chain protein (NfL), glial fibrillary acidic protein (GFAP) and total Tau (t-Tau), in CSF from 127 patients with psychiatric disease preselected for suspected immunological involvement were related to recently proposed clinical red flags, psychiatric features, and MRI and EEG findings. Twenty-one percent had abnormal routine CSF findings and 27% had elevated levels of CNS damage markers. Six percent had anti-neuronal antibodies in serum and 2% had these antibodies in the CSF. Sixty-six percent of patients examined with MRI (n = 88) had alterations, mostly atrophy or nonspecific white matter lesions. Twenty-seven percent of patients with EEG recordings (n = 70) had abnormal findings. Elevated NfL levels were associated with comorbid autoimmunity and affective dysregulation symptoms. Elevated t-Tau was associated with catatonia and higher ratings of agitation/hyperactivity. Elevated GFAP was associated with acute onset, atypical presentation, infectious prodrome, tics, depressive/anxiety symptom ratings and overall greater psychiatric symptom burden. In conclusion, preselection based on suspected autoimmune psychiatric disease identifies a population with a high prevalence of CSF alterations suggesting CNS pathology. Future studies should examine the value of these markers in predicting treatment responses.

Orthogonality of sensory and contextual categorical dynamics embedded in a continuum of responses from the second somatosensory cortex

How does the brain simultaneously process signals that bring complementary information, like raw sensory signals and their transformed counterparts, without any disruptive interference? Contemporary research underscores the brain's adeptness in using decorrelated responses to reduce such interference. Both neurophysiological findings and artificial neural networks support the notion of orthogonal representation for signal differentiation and parallel processing. Yet, where, and how raw sensory signals are transformed into more abstract representations remains unclear. Using a temporal pattern discrimination task in trained monkeys, we revealed that the second somatosensory cortex (S2) efficiently segregates faithful and transformed neural responses into orthogonal subspaces. Importantly, S2 population encoding for transformed signals, but not for faithful ones, disappeared during a nondemanding version of this task, which suggests that signal transformation and their decoding from downstream areas are only active on-demand. A mechanistic computation model points to gain modulation as a possible biological mechanism for the observed context-dependent computation. Furthermore, individual neural activities that underlie the orthogonal population representations exhibited a continuum of responses, with no well-determined clusters. These findings advocate that the brain, while employing a continuum of heterogeneous neural responses, splits population signals into orthogonal subspaces in a context-dependent fashion to enhance robustness, performance, and improve coding efficiency.

A spatial map: a propitious choice for constraining the binding problem.

Many studies have shown that the human visual system has two major functionally distinct cortical visual pathways: a ventral pathway, thought to be important for object recognition, and a dorsal pathway, thought to be important for spatial cognition. According to our and others previous studies, artificial neural networks with two segregated pathways can determine objects' identities and locations more accurately and efficiently than one-pathway artificial neural networks. In addition, we showed that these two segregated artificial cortical visual pathways can each process identity and spatial information of visual objects independently and differently. However, when using such networks to process multiple objects' identities and locations, a binding problem arises because the networks may not associate each object's identity with its location correctly. In a previous study, we constrained the binding problem by training the artificial identity pathway to retain relative location information of objects. This design uses a location map to constrain the binding problem. One limitation of that study was that we only considered two attributes of our objects (identity and location) and only one possible map (location) for binding. However, typically the brain needs to process and bind many attributes of an object, and any of these attributes could be used to constrain the binding problem. In our current study, using visual objects with multiple attributes (identity, luminance, orientation, and location) that need to be recognized, we tried to find the best map (among an identity map, a luminance map, an orientation map, or a location map) to constrain the binding problem. We found that in our experimental simulations, when visual attributes are independent of each other, a location map is always a better choice than the other kinds of maps examined for constraining the binding problem. Our findings agree with previous neurophysiological findings that show that the organization or map in many visual cortical areas is primarily retinotopic or spatial.

Trauma-informed approach for bridging ethnic distance in post-conflict societies

The article presents a pilot study based on intervention conducted in Croatia in 2022. Despite intensive peacebuilding efforts, ethnic tensions in post-conflict societies remain high, suggesting that current strategies may be missing critical components. This experiment integrated recent neurophysiological findings, particularly polyvagal theory, into peacebuilding intervention. The intervention involved a two-day seminar in Zagreb, where trauma-informed, body-based techniques were used to address post-war trauma. Participants learnt to recognise and manage trauma reactions by promoting their interoceptive awareness and a felt sense of safety. The seminar included both theoretical and experiential components, focusing on the impact of trauma on the autonomous nervous system and interpersonal dynamics. The study used an experimental design with intervention and control group to investigate the effects of the seminar. Measures included interoceptive awareness, autonomic reactivity, ethnic distance, perspective taking and mental well-being. The results showed that the participants in the intervention group showed significant improvements in interoceptive awareness, particularly in not worrying and emotional awareness subscales compared to the control group. However, ethnic distance scores did not decrease as expected, highlighting the complexity of changing deep-rooted attitudes. The study faced some challenges, including participant recruitment and the short duration of the intervention, which may have limited its effectiveness. Despite these limitations, the findings suggest that integrating trauma-informed approaches into peacebuilding efforts holds promise for improving interethnic relations. Future research should focus on longer interventions and diverse participant groups to better understand the potential of these methods in various contexts.

Disentangling Bradykinesia and Rigidity in Parkinson's Disease: Evidence from Short- and Long-Term Subthalamic Nucleus Deep Brain Stimulation.

Bradykinesia and rigidity are considered closely related motor signs in Parkinson disease (PD), but recent neurophysiological findings suggest distinct pathophysiological mechanisms. This study aims to examine and compare longitudinal changes in bradykinesia and rigidity in PD patients treated with bilateral subthalamic nucleus deep brain stimulation (STN-DBS). In this retrospective cohort study, the clinical progression of appendicular and axial bradykinesia and rigidity was assessed up to 15 years after STN-DBS in the best treatment conditions (ON medication and ON stimulation). The severity of bradykinesia and rigidity was examined using ad hoc composite scores from specific subitems of the Unified Parkinson's Disease Rating Scale motor part (UPDRS-III). Short- and long-term predictors of bradykinesia and rigidity were analyzed through linear regression analysis, considering various preoperative demographic and clinical data, including disease duration and severity, phenotype, motor and cognitive scores (eg, frontal score), and medication. A total of 301 patients were examined before and 1 year after surgery. Among them, 101 and 56 individuals were also evaluated at 10-year and 15-year follow-ups, respectively. Bradykinesia significantly worsened after surgery, especially in appendicular segments (p < 0.001). Conversely, rigidity showed sustained benefit, with unchanged clinical scores compared to preoperative assessment (p > 0.05). Preoperative motor disability (eg, composite scores from the UPDRS-III) predicted short- and long-term outcomes for both bradykinesia and rigidity (p < 0.01). Executive dysfunction was specifically linked to bradykinesia but not to rigidity (p < 0.05). Bradykinesia and rigidity show long-term divergent progression in PD following STN-DBS and are associated with independent clinical factors, supporting the hypothesis of partially distinct pathophysiology. ANN NEUROL 2024;96:234-246.

Mapping network connection and direction among symptoms of depression and anxiety in patients with chronic gastritis.

Regarding neurophysiological and developmental findings, anxiety and depression are usual comorbidities of gastritis patients. However, research related to anxiety and depression among chronic gastritis patients was conducted on the disease level while ignoring symptoms. Hence, we rendered the network approach to reveal the symptoms of anxiety and depression among chronic gastritis patients. Three hundred and sixty-nine chronic gastritis patients (female = 139, Mage = 55.87 years) were asked to complete the Self-Rating Anxiety Scale and Self-Rating Depression Scale. Three symptom networks and one directed acyclic graph (DAG) network were formed. First, in the anxiety network of chronic gastritis patients, dizziness was the most influential symptom. In the depression network of chronic gastritis patients, depressed affect and psychomotor retardation were the influential symptoms. Second, panic, easy fatiguability, weakness, palpitation, depressed affect, tachycardia, fatigue, and psychomotor agitation bridged the anxiety-depression network of chronic gastritis patients. Third, DAG networks showed that anxiousness and hopelessness could trigger other symptoms in the anxiety-depression networks of chronic gastritis patients. The current study provided insightful information on patients with chronic gastritis by examining the structures of symptoms.

Alcohol-related peripheral neuropathy: Clinico-neurophysiological characteristics and diagnostic utility of the neuropathy symptoms score and the neuropathy impairment score

Alcohol overconsumption is well known to cause damage to the peripheral nervous system, affecting both small and large nerve fibers. The aim of this descriptive study was to investigate peripheral nerve damage, and to correlate clinical, epidemiological and neurophysiological findings, in patients diagnosed with Alcohol Use Disorder (AUD). Ninety alcohol-dependent subjects on inpatient basis were enrolled in this prospective study over a 3-year period. Every subject was assessed by the Neuropathy Symptoms Score (NSS) questionnaire and the Neuropathy Impairment Score (NIS) clinical examination grading scale, followed by Nerve Conduction Studies, Quantitative Sensory Testing and Sympathetic Skin Response (SSR) testing. Peripheral neuropathy was diagnosed in 54 subjects (60%), by abnormal neurophysiological tests and presence of clinical signs or symptoms. Among them, pure large fiber neuropathy (LFN) was found in 18 subjects, pure small fiber neuropathy (SFN) in 12 subjects, and both large and small fiber neuropathy was diagnosed in 24 subjects. Using linear regression, we found that higher NSS and NIS scores correlated with lower amplitudes of the sural sensory nerve action potential and of the SSR. We also found a significant longer duration of alcohol abuse in subjects with neuropathy, using Student's t-test (p = 0.024). Additionally, applying NIS abnormal cut-off score ≥4, using ROC analysis, we predicted the majority of subjects with LFN, confirming 95.23% sensitivity and 93.75% specificity. Our study confirmed that peripheral neuropathy involving large and small nerve fibers, with a symmetrical length-dependent pattern, is common between patients with AUD and related to the duration of the disorder. We suggest that NSS and NIS scales could be used for the assessment of neuropathy in clinical practice, when the essential neurophysiological testing is not available.

Pacientų, turinčių celiakijos sukeltų neurologinių simptomų, diagnostikos ir gydymo galimybės

Celiac disease (CD) is a gastrointestinal disorder caused by the immune system’s response to gluten, involving both innate and adaptive immune reactions. It leads to various neurological issues, such as gluten ataxia, gluten neuropathy, epilepsy, and gluten encephalopathy. Although neurological presentations are rare in children, they are observed in up to 36% of adult patients with CD. Recent evidence suggests that these manifestations may be linked to gluten-related mechanisms, including antibody cross-reactions, immune-complex deposition, direct neurotoxicity, and, in severe cases, vitamin or nutrient deficiencies. However, there is still no consensus on whether serological, neurophysiological, or neuroimaging findings can effectively diagnose and monitor CD-associated neurological problems at an early stage. The identification of multimodal biomarkers and suitable neuroimaging tools could aid in the diagnosis, monitoring, and enhancement of the quality of life for individuals with neuroceliac disease. Nonetheless, it is essential to provide appropriate treatment to those with CD and neurological symptoms, as prolonged suffering may lead to irreversible disability. The primary treatment for neurological manifestations of gluten-related disorders is a strict gluten-free diet, although a small number of patients may require additional immunosuppressive therapy, typically using mycophenolate or intravenous immunoglobulins. In this literature review, our aim was to explore the relevant neurological disorders associated with CD, early diagnostic and treatment options to prevent related disability in affected patients. Clinicians should consider CD as a potential cause in individuals presenting with unexplained neurological dysfunction.

Determining the Algorithm of Rehabilitation Procedures in Patients with Brachial Plexus Injuries Based on the Prospective Single-Centre Clinical Neurophysiology Studies: Preliminary Results

The clinical neurophysiological tests allow us to determine the type, extent, and nature of brachial plexus damage. They are crucial in decision making regarding surgical procedures or conservative treatment. This report aimed to present an algorithm for rehabilitation procedures in patients with brachial plexus injury of various origins based on the results of neurophysiology findings for the selection of procedures supporting the process of nerve and muscle regeneration. The research group consisted of patients whose medical documentation was analysed concerning the reason, level, and localization of damage to the brachial plexus structures, surgical and or rehabilitative treatment, as well as the MRI results. Among the group of fourteen patients, the clinical studies showed the greatest incidence of brachial plexus injuries of the mixed (both pre- and postganglionic), all trunks, and cervical root injuries, respectively. Results of the motor evoked potentials (MEP) and electroneurography (ENG) recordings induced at levels of spinal roots and Erb’s point showed a decrease of more than 40% in amplitudes on the symptomatic side in comparison to the asymptomatic side. This diffeence was recorded for the axillary and radial innervation and the C5, C6, and C7 root domains, ranging from 57% to 66%; the lowest decrease was recorded following electrical stimulation at Erb’s point for the ulnar nerve (34%). The latency prolongation on the symptomatic side in CMAP and MEP tests ranged from 0.2 to 1.7 ms, with the most following magnetic stimulation of the C5 cervical root for the axillary innervation. Most of the results indicated the axonotmesis and neuropraxia type of injury in motor fibers (40%) confirmed by EMG results. The sensory conduction studies (SNCS) in distal nerve branches did not confirm the severe advancement of the brachial plexus injury (63%). The proposed algorithm of the physiotherapeutic procedures should be mainly targeted for recovery of motor dysfunction as the consequence of brachial plexus injury. Rehabilitation should incorporate the treatment supporting nerve regeneration, muscle strengthening, and maintaining functional ranges of motion of the injured extremities. The rehabilitation treatment for patients with brachial plexus injuries is an individualised process, and the selection of procedures and the effectiveness of the treatment undertaken should be confronted with results of neurophysiological tests verifying the motor neural transmission from the level of the cervical motor centre to the effector, peripheral nerve function, and muscle’s motor unit activity.

Relationship between Gravidity, Parity, and Neurophysiological Features in Carpal Tunnel Syndrome (CTS) Patients with a History of Pregnancy.

It is not yet clear if gravidity and parity have any relationships with the electrodiagnostic parameters of carpal tunnel syndrome (CTS). To determine whether there is a relationship between electrodiagnostic findings, gravidity, and parity number in CTS. Female patients over 18 years of age with CTS were included in this retrospective cohort study. The gravidity/parity number, median nerve compound muscle action potential (CMAP), and compound nerve action potential (CNAP) of the patients were analyzed. The two subgroups of the Boston carpal tunnel syndrome questionnaire (BCTSQ): The Symptom Severity Scale (SSS) and Functional Severity Scale (FSS) were applied to the patients. CTS patients were divided into two groups aged at first pregnancy ≤20 years and >20 years. One hundred and eight CTS extremities (seven right-sided CTS, three left-sided CTS, 49 bilateral CTS) of 59 patients were included. The median (interquartile range: 25%-75%) number of gravidity, parity, and abortion were 3 (2-5), 3 (2-4), and 0 (0-0), respectively. Right-sided CTS patients at the age of first pregnancy ≤20 years had higher BCTSQ-SSS/FSS scores and median nerve CMAP latency compared to patients at the age of first pregnancy >20 years (P = 0.029 for SSS; P = 0.042 for FSS; and P = 0.041 for CMAP latency). A negative correlation was found between the gravidity/parity numbers and median nerve CNAP/CMAP amplitudes (P = 0.028/0.031, r = -0.293/-0.289 for CNAP amplitude; and P = 0.006/0.035, r = -0.363/-0.283 for CMAP amplitude). Neurophysiological findings worsen as the number of gravidity and parity increase. Electrodiagnostic and clinical features of CTS may be worsening in females below 20 years at first pregnancy.