Unilateral Atrophy Research Articles

Pregnancy and delivery after functional hemispherectomy for Rasmussen’s encephalitis: a case report

BackgroundRasmussen’s encephalitis (RE) is a rare neurologic disorder characterized by progressive seizures and unilateral cerebral atrophy with onset during childhood and unknown etiology. When medical therapy appears refractory, surgical disconnection of the affected hemisphere is indicated. Quality of life after functional hemispherectomy is largely good, affected females may therefore pursue pregnancy. However, data on pregnancy and delivery in RE post hemispherectomy is extremely rare.Case presentationWe present the case of a patient with left functional hemispherectomy for RE at the age of seven, who experienced two successful pregnancies. In both pregnancies, her post-surgical symptoms including right-sided spasticity, cephalgia, dizziness, and impairment of vision and speech deteriorated but improved to pre-pregnancy level after delivery. Neurologic sequelae post-hemispherectomy overlapped with clinical signs of preeclampsia and required close diagnostic surveillance during both pregnancies.ConclusionThere are no data on the interaction between RE, hemispherectomy and pregnancy, making maternal and fetal risk assessment difficult. Due to the complexity of the condition and symptoms, management of RE in pregnancy remains highly challenging and requires an interdisciplinary approach. This is the first case description of two successful pregnancies in a woman with RE and status post-hemispherectomy. Further evidence is urgently required to improve counseling and management of affected women.

A Wearable Solution of Muscle Atrophy Assessment: Oriented Toward Upper Limb Rehabilitation

In the process of the upper limb rehabilitation, the rehabilitation effect is often evaluated from the perspective of the motor function of limbs. However, the state of muscle atrophy is also a noteworthy indicator reflecting the rehabilitation effect. We proposed a wearable solution for the monitoring and grade assessing of local muscle atrophy based on wearable bioimpedance (BioZ) sensors. This work elaborates on the theoretical basis, procedure, and key influencing factors of the proposed solution, and the feasibility and effectiveness have been verified through in vitro and in vivo experiments. A total of 25 phantoms in different CSA (cross-sectional area) and FMR (fat-to-muscle ratio) values were designed to simulate different stages of muscular atrophy, and a linear correlation was observed between BioZ, CSA, and FMR, with an R-squared value of 0.898. The relative impedance difference of 38 patients with unilateral muscle atrophy was 5.231% larger than that of 30 healthy control samples on average (p< 0.05). These results demonstrate the correlation between muscle atrophy and BioZ. As the proof-of-concept for graded assessment, the results analyzed by support vector machines (SVMs) show that the accuracy of three-level classification can reach 94.1% using the five-fold cross-validation.

Long Term Follow Up of a Family with A3243G Mitochondrial Syndrome.

To describe the clinical and multimodal imaging (MMI) features of a family (proband, sister and mother) with A3243G mitochondrial retinopathy and long-term follow up. Medical and imaging records were retrospectively evaluated. Multimodal imaging included ultra-widefield color fundus photography, fundus autofluorescence, and spectral-domain optical coherence tomography. Long-term MMI follow up ranged from 6 to 15 years and was available for each member. The proband (Case 1) exhibited rapidly progressive bilateral macular atrophy, corneal endothelial polymegathism, and an A3243G mutation in the mitochondrial DNA. The proband also endorsed a history of early-onset myocardial infarction (MI) ten years prior at the age of 42. The proband's sister (Case 2) only exhibited unilateral focal macular atrophy but admitted to a history of severe multiorgan systemic disease, including multiple sclerosis and major depression disorder. The proband's mother (Case 3), the only one with diabetes mellitus and hearing loss, originally presented with branch retinal vein occlusion in the right eye and pattern dystrophy in the left eye which evolved to geographic atrophy, OD > OS, 15 years later. A3243G mitochondrial syndrome can exhibit heterogenous ocular and systemic features, even within members of the same family. The development of the characteristic maculopathy with early-onset MI warrants genetic testing. Early cardiac and systemic screening may be recommended in individuals with characteristic retinal findings and genetic confirmation.

Unilateral Trapezius Atrophy in a College Baseball Player: A Clinical Vignette.

Unilateral Trapezius Atrophy in a College Baseball Player: A Clinical Vignette.

Collet-Sicard syndrome due to cervical artery dissection disclosed by high-resolution magnetic resonance imaging.

Cervical artery dissection (CAD) represents a leading cause of unilateral lower cranial nerve IX-XII palsy, known as Collet-Sicard syndrome (CSS). High-resolution magnetic resonance imaging (HR-MRI) is widely used in the evaluation of patients with CAD, providing information regarding vessel wall abnormalities and intraluminal thrombus. We present a patient with palsy of multiple lower cranial nerves in the context of CSS, attributed to unilateral spontaneous internal carotid artery dissection. We describe a 68-year-old man with unremarkable previous history, who presented with subacute, gradually worsening dysphagia and hoarse voice. Clinical examination revealed right-sided palsy of cranial nerves IX-XII. Three-dimensional fat-saturated black-blood T1-weighted high-resolution vessel wall imaging disclosed spontaneous dissection with intramural hematoma along the distal right internal carotid artery. Neck MRI showed inward displacement of right aryepiglottic fold, right pyriform sinus dilatation, and right true vocal cord in middle position, indicative of right vagus nerve palsy, atrophy of right trapezius and sternocleidomastoid muscles, due to right spinal accessory nerve palsy, and unilateral tongue atrophy with fatty infiltration, characteristic for right hypoglossal nerve palsy. This case highlights the utility of high-resolution vessel wall imaging and especially fat-saturated T1-weighted black-blood SPACE (sampling perfection with application-optimized contrast using different flip-angle evolutions) sequences in the accurate diagnosis of CAD, revealing the characteristic mural hematoma and intimal flap. HR-MRI is also valuable in the recognition of indirect signs of lower cranial nerve compression.

Transcutaneous Laryngeal Ultrasound for Assessing Hyaluronic Acid Status in Patients Undergoing Injection Laryngoplasty.

Injection laryngoplasty (IL) with hyaluronic acid (HA) is an effective treatment for patients with glottic insufficiency. The duration of HA maintenance in the vocal fold remains unknown. In this study, transcutaneous laryngeal ultrasound (TLUS) was used to evaluate the absorption and migration of HA after IL. Subsequent management might be provided based on the TLUS finding. Patients diagnosed with unilateral vocal fold paralysis (UVFP) or vocal fold atrophy were recruited. All patients underwent IL with HA in an office-based setting along with TLUS to monitor the status of HA. The schedule of TLUS included assessments before and after IL until non-visualization. The study population comprised 38 women and 17 men. Of the patients, 54.1% underwent IL for UVFP, whereas 45.9% underwent IL for vocal fold atrophy. Multivariate Cox regression analysis for factors affecting HA absorption revealed that the cause of injection was the most important independent predictor (hazard ratio [HR], 2.15; 95% confidence interval [CI], 1.03-4.46; p = 0.040). The duration of HA maintenance was significantly longer in patients with UVFP than in those with vocal fold atrophy (8.77 vs. 4.70 months, HR, 2.33; 95% CI, 5.47-8.18; p = 0.002). TLUS is an objective assessment method for patients undergoing IL with HA. Subsequent tailor-made management could be offered based on the TLUS findings during follow-up. For patients at high risk of upper respiratory tract infection or who are intolerant to flexible nasopharyngoscopy, TLUS can be used as an alternative tool to evaluate the condition of the glottis after IL with HA. 4 Laryngoscope, 134:4682-4687, 2024.

Perimacular Atrophy Following Voretigene Neparvovec-Rzyl Treatment in the Setting of Previous Contralateral Eye Treatment With a Different Viral Vector.

To report on cases of unilateral perimacular atrophy after treatment with voretigene neparvovec-rzyl, in the setting of previous contralateral eye treatment with a different viral vector. Single-center, retrospective chart review. In this case series, four patients between the ages of six and 11years old with RPE65-related retinopathy were treated unilaterally with rAAV2-CB-hRPE65 as part of a gene augmentation clinical trial (NCT00749957). Six to 10 years later the contralateral eyes were treated with the Food and Drug Administration-approved drug, voretigene neparvovec-rzyl. Best-corrected visual acuity (BCVA), fundus photos, ocular coherence tomography, two-color dark-adapted perimetry, full field stimulus threshold testing (FST), and location of subretinal bleb and chorioretinal atrophy were evaluated. Three out of four patients showed unilateral perimacular atrophy after treatment with voretigene, ranging from five to 22months after treatment. Areas of robust visual field improvement were followed by areas of chorioretinal atrophy. Despite perimacular changes, BCVA, FST, and subjective improvements in vision and nyctalopia were maintained. Perimacular atrophy was not observed in the first eye treated with the previous viral vector. We observed areas of robust visual field improvement followed by perimacular atrophy in voretigene treated eyes, as compared to the initially treated contralateral eyes. Caution is advised when using two different viral vectors between eyes in gene therapy. This may become an important issue in the future with increasing gene therapy clinical trials for inherited retinal dystrophies.

Osteoarthritis of the coxofemoral joint in 24 horses: Evaluation of radiography, ultrasonography, intra-articular anaesthesia, treatment and outcome.

Few case reports describe equine coxofemoral joint osteoarthritis (CFJOA). To evaluate diagnostic findings and outcome of horses with CFJOA and to provide a score facilitating radiographic assessment. Retrospective case series. History, clinical signs, ultrasonographic, radiographic and intra-articular anaesthesia findings, treatments, outcome, and necropsy results of horses with CFJOA presented between 2002 and 2023 were collated. Radiographic findings were categorised to develop a radiographic score which was applied by two masked examiners. Intra- and inter-observer reliability was determined using weighted Cohen's kappa (Cκ) and the correlation between radiographic and ultrasound findings via Spearman correlation coefficient. The study included 24 horses (median age 14 years). Most of them (20/24) were chronically lame. Frequent clinical signs included unilateral gluteal muscle atrophy (18/21), lengthening of the stride of the affected limb (13/19) and locomotion on three tracks (13/20). Both imaging modalities enabled evaluation of periarticular osteophytes (correlation coefficient r = 0.64; p = 0.003). Additionally, radiography allowed detection of irregular joint spaces, subchondral bone opacity changes and femoral head flattening/tapering. Inter-observer (Cκ = 0.846) and intra-observer (Cκ = 0.853 and Cκ = 0.842) agreement was excellent. If treated, mostly intra-articular corticosteroids were administered (16/18). Nine horses were euthanised immediately or during follow-up examination. Post-mortem, the Ligamentum capitis ossis femoris was commonly found ruptured. All surviving horses remained lame. Retrospective analysis of clinical records and subjective outcome assessment based on owner follow-up with potential recall bias. Due to overall disease severity, associations between different grades of clinical findings, radiographic abnormalities and outcome could not be evaluated. Typical clinical signs may indicate CFJOA. Standardised evaluation of ventrodorsal radiographs allows a comprehensive diagnosis. Postmortem findings suggest joint instability as a possible causative factor that may contribute to the poor prognosis and resistance to medical therapy of the disorder.

Diagnosis and treatment of 11 patients with cevical spondylotic amyotrophy

To explore clinical features, treatment methods and clinical effects of cervical spondylosis with proximal muscular atrophy. Eleven patients with proximal-type cervical spondylotic amyotrophy were retrospectively studied from September 2016 to November 2020, including 7 males and 4 females, aged 38 to 68 years old. Clinical symptoms, MRI and neuroelectrophysiological manifestations were analyzed, and patients were treated with conservative treatment or anterior cervical decompression fusion surgery, respectively. The efficacy was evaluated by manual muscle test (MMT) before and after treatment, and patients' satisfaction was followed up at the same time. All patients were followed up for 6 to 19 months. All 11 patients were unilateral, mainly manifested by atrophy of deltoid muscle, supraspinatus muscle and infraspinatus muscle, and may be accompanied by ipsilateral neck and shoulder pain at early stage. MRI showed lesions at C4,5, C5,6 segments were more common. Electrophysiological examination showed the affected muscle was denervated, and amplitude of compound muscle action potential (CMAP) of innervated nerve on the affected side was lower than that on the healthy side. All patients were obtained bone fusion. One patient who were underwent anterior cervical corpectomy and fusion (ACCF) occurred developed contralateral C5 nerve root paralysis after operation, which recovered completely after 10 weeks of symptomatic treatment. At 12 months after operation, the efficacy was evaluated according to MMT, 3 patients were treated conservatively, 2 patients excellent and 1 good;in 8 patients treated by operation, 3 patients were excellent, 4 good, and 1 moderate. The incidence of cervical spondylosis with proximal muscular atrophy is low, which is manifested as unilateral proximal muscle atrophy and may be accompanied by ipsilateral neck and shoulder pain in the early stage. Combined with MRI and neuroelectrophysiological examination, misdiagnosis could be reduced. In the early stage of disease, especially in the case of nucleus pulposus protrusion leading to nerve compression, conservative treatment could be taken. When the conservative treatment is ineffective or the pain cannot be tolerated, anterior decompression surgery is recommended, and the overall effect is satisfactory.

Transcriptome Sequencing of the Oral Mucosa Reveals Gene Expression Differences in Patients With Parry-Romberg Syndrome

Objective: Parry-Romberg syndrome (PRS) is an acquired disease characterized by progressive unilateral atrophy of the facial skin, subcutaneous tissue, muscle, and bone. There are various hypotheses to try to explain the occurrence of the disease, but the specific etiology and pathogenesis remain unclear. This study aimed to explore the potential molecular pathogenesis of the disease by using next-generation RNA-sequencing technology. Methods: The authors collected oral mucosal tissue from the affected side and the healthy side from 3 patients with PRS. Tissue samples were subjected to RNA extraction, whole transcriptome sequencing, and bioinformatics analysis. Differentially expressed genes were obtained from both groups of samples and then analyzed for functional enrichment. Results: A total of 186 differentially expressed genes were screened from the 2 groups of samples. Compared with the healthy side, several immune-related genes, including immunoglobulin kappa variable (IGKV)2D-28, IGKV1D-33, IGKV1-33, and NLRP10, were significantly upregulated in the affected tissue. In addition, the differential genes were significantly enriched in metabolic pathways including pancreatic secretion, protein and fat digestion, and absorption. Conclusions: The authors described the gene expression differences between the affected and healthy tissues of patients with PRS for the first time. Immune responses may play a role in the pathogenesis of PRS.

Globally altered microstructural properties and network topology in Rasmussen's encephalitis.

Rasmussen's encephalitis is an immune-mediated brain disorder characterised by progressive unilateral cerebral atrophy, neuroinflammation, drug-resistant seizures and cognitive decline. However, volumetric changes and epileptiform EEG activity were also observed in the contralateral hemisphere, raising questions about the aetiology of contralateral involvement. In this study, we aim to investigate alterations of white matter integrity, structural network topology and network efficiency in Rasmussen's encephalitis using diffusion-tensor imaging. Fourteen individuals with Rasmussen's encephalitis (11 female, median onset 6 years, range 4-22, median disease duration at MRI 5 years, range 0-42) and 20 healthy control subjects were included. All subjects underwent T1-weighted structural and diffusion-tensor imaging. Diffusion-tensor images were analysed using the fixel-based analysis framework included in the MRtrix3 toolbox. Fibre density and cross-section served as a quantitative measure for microstructural white matter integrity. T1-weighted structural images were processed using FreeSurfer, subcortical segmentations and cortical parcellations using the Desikan-Killiany atlas served as nodes in a structural network model, edge weights were determined based on streamline count between pairs of nodes and compared using network-based statistics. Global efficiency was used to quantify network integration on an intrahemispheric level. All metrics were compared cross-sectionally between individuals with Rasmussen's encephalitis and healthy control subjects using sex and age as regressors and within the Rasmussen's encephalitis group using linear regression including age at onset and disease duration as independent variables. Relative to healthy control subjects, individuals with Rasmussen's encephalitis showed significantly (family-wise-error-corrected P < 0.05) lower fibre density and cross-section as well as edge weights in intrahemispheric connections within the ipsilesional hemisphere and in interhemispheric connections. Lower edge weights were noted in the contralesional hemisphere and in interhemispheric connections, with the latter being mainly affected within the first 2 years after disease onset. With longer disease duration, fibre density and cross-section significantly (uncorrected P < 0.01) decreased in both hemispheres. In the contralesional corticospinal tract, fibre density and cross-section significantly (uncorrected P < 0.01) increased with disease duration. Intrahemispheric edge weights (uncorrected P < 0.01) and global efficiency significantly increased with disease duration in both hemispheres (ipsilesional r = 0.74, P = 0.001; contralesional r = 0.67, P = 0.012). Early disease onset was significantly (uncorrected P < 0.01) negatively correlated with lower fibre density and cross-section bilaterally. Our results show that the disease process of Rasmussen's encephalitis is not limited to the cortex of the lesioned hemisphere but should be regarded as a network disease affecting white matter across the entire brain and causing degenerative as well as compensatory changes on a network level.

Twenty Years of Managed Epilepsy for a Stranded Male Guadalupe Fur Seal (Arctocephalus townsendi) Secondary to Suspect Domoic Acid Toxicosis

Many wild otariids (sea lions and fur seals) strand along the California coast annually with seizures following presumed exposure to harmful algal blooms (HABs). This is a long-term case study of a stranded subadult male Guadalupe fur seal (Arctocephalus townsendi) that stranded in 2001 and within days after admission began demonstrating seizure activity. The epilepsy was stabilized with antiepileptic drug (AED) therapy and advanced imaging was performed. Magnetic resonance (MR) scans showed signs of encephalopathy consistent with domoic acid (DA) toxicosis. This fur seal was deemed “non-releasable” and over the following 19 years, he received supportive veterinary care and AED treatment at a permitted facility. In the summer of 2020, the fur seal showed progressive behavioral and functional deterioration whereupon humane euthanasia was performed. Post-mortem MR and diffusion tensor images (DTI) were acquired. A volumetric comparison with historic scans showed evidence of severe progressive unilateral right hippocampal atrophy. Histopathology confirmed severe chronic right and mild left hippocampal atrophy. The progressive degenerative brain changes demonstrate how adaptive brain function can be with neuronal atrophy secondary to DA exposure. This case highlights some considerations and decision-making processes needed for the rehabilitation and placement of wild stranded otariids with acquired epilepsy secondary to DA toxicity.

The Important Role of Hemispherotomy for Rasmussen Encephalitis: Clinical and Functional Outcomes

BackgroundRasmussen encephalitis (RE) is characterized by pharmacoresistant epilepsy and progressive neurological deficits concurrent with unilateral hemispheric atrophy. Evidence of an inflammatory autoimmune process has been extensively described in the literature; however, the precise etiology of RE is still unknown. Despite data supporting a beneficial effect of early immunosuppressive and immunomodulatory interventions, surgical disconnection of the affected hemisphere is considered the treatment of choice for these patients. The aim of this study was to report a series of children and adolescents who underwent hemispheric surgery (HS) for the treatment of RE, analyzing their clinical, electrographic, and neuroimaging features pre-operatively, as well as their postoperative status, including seizure and functional outcomes. MethodsAll patients with RE who underwent HS in the Epilepsy Surgery Center (CIREP) of the University Hospital of Ribeirão Preto Medical School, between 1995 and 2020 were retrospectively reviewed. Preoperative and postoperative analyses included gender; age at epilepsy onset; seizure semiology; seizure frequency; interictal and ictal electroencephalographic (EEG) findings; age at surgery; duration of epilepsy; surgical complications; duration of follow-up; histopathological findings; and postoperative seizure, cognitive, and functional outcomes. ResultsForty-four patients were evaluated. Mean age at seizure onset and epilepsy duration was 6 years and 2.5 years, respectively. Mean age at surgery was 9 years, with an average follow-up ranging from 3 months to 23 years. All patients presented with severe epilepsy and distinct neurological abnormalities on MRI. Before HS, different degrees of abnormal intellectual performance as well as hemiparesis were seen in 86% and 90%, respectively. Histopathology examination confirmed this diagnosis in 95% patients. At the last follow-up, 68% of patients were seizure free, and 70% were classified as Engel Class I or II. Postoperatively, the cognitive status remained unchanged in 64% of patients. Likewise, the gross motor function remained unchanged in 54% of patients and 74% had functional hand ability after HS. ConclusionsConsidering the progressive damage course of RE, hemispheric surgery should be offered to pediatric patients. It has manageable risks and results in good seizure outcome, and the preoperative functional status of these children is often preserved (even when the left hemisphere is involved), thus improving their quality of life.

P08 Appearance matters: a case of adult-onset Parry-Romberg syndrome with linear scleroderma en coup de sabre and neurological involvement

Abstract Introduction We describe the case of an adult patient who initially presented with neurological symptoms. Investigations were suggestive of a neuroinflammatory disorder but the diagnosis was unclear. It was only as time passed that the cutaneous signs and symptoms of Parry-Romberg syndrome (PRS) and linear scleroderma en coup de sabre (LSCS) became apparent to clinicians as the underlying cause for her presentation. Case description This female patient, with no past medical history, presented in 2015 when she was 43 years old. She reported a one-day history of malaise and lethargy, left-sided headache, numbness affecting the right face and right arm and dysphasia. Her symptoms gradually improved over the following few days. An MRI brain showed extensive T2 hyperintensity in the right frontal and frontoparietal regions. Cerebrospinal fluid (CSF) showed a lymphocytosis and oligoclonal bands, which were present in the CSF only. A repeat MRI brain a few weeks later showed slightly less extensive changes. This was treated by neurologists as a neuroinflammatory disorder with intravenous methylprednisolone pulses. At follow-up, she mentioned that she had noticed a change in her appearance, with the right side of her face becoming increasingly asymmetrical over the last few years. Neurologists were puzzled regarding the diagnosis, but given that she was almost asymptomatic at review, they decided to monitor her with serial MRI scans. There were no further neurological episodes and no interval changes on repeat scans. In 2020, she was referred to ophthalmology, as she had noticed that her right eye had become increasingly sunken. MRI of the orbits and sinuses was reported to show enophthalmos of no obvious cause. Around the same time, she was also referred to dermatology for an enlarging patch of alopecia over the right frontoparietal scalp. Autoimmune screening blood tests including ANA, ENA, dsDNA and centromere were negative. Scalp biopsy was inconclusive but was most in keeping with old alopecia areata. She was treated with intralesional steroids and had a partial response. It was only by 2021 that PRS with LSCS was recognised as the cause of her symptoms. We can be confident that her neuroinflammation is an extracutaneous manifestation, which occurred early on when cutaneous signs were subtle. Discussion PRS is a rare, acquired disorder characterised by progressive unilateral atrophy of facial skin, soft tissue, muscles, and underlying bony structures. LSCS is a subtype of linear scleroderma that occurs on the forehead or frontoparietal scalp. PRS and LSCS commonly coexist, and it is debated whether they are on a spectrum of the same disease. Both usually occur in childhood or adolescence, and adult-onset disease is very rare. The diagnoses are clinical and there are no specific diagnostic tests. Both are associated with neurological involvement, including seizures, headaches, focal neurological deficits and neuropsychiatric changes. Usually, the cutaneous lesion develops months to years before the onset of neurological symptoms. Our patient had noticed subtle changes in her appearance at least five years before her presentation in 2015. Neuroimaging abnormalities are predominately ipsilateral to the cutaneous lesions but may rarely be contralateral. Key learning points Given that adult-onset PRS and LSCS are very rare, there may be a lack of awareness of the conditions amongst adult physicians. This can lead to diagnostic delay. This case highlights that PRS and LSCS are not just conditions of the skin and connective tissues and that neurological involvement is a frequent extracutaneous manifestation. This case is also a reminder of the importance of a thorough examination of the skin, especially when the diagnosis is unclear.

A case of progressive crossed hemiatrophy mistaken as panniculitis.

Progressive crossed hemiatrophy is an extremely rare clinical type of facial hemiatrophy that presents primarily as unilateral facial atrophy and contralateral trunk or limb involvement. The undistinguishable clinical manifestation and pathological changes complicate diagnosis, especially at the onset of the disease when presenting with less clinical evidence. Here, we report a case of a 9-year-old boy started with left scalp induration, following with subcutaneous tissues atrophy on the right trunk. He was mistaken as panniculitis based on the pathologic findings and treated with topical tacrolimus without any improvement. Immune-related tests were implemented to exclude connective tissues. Imaging examinations such as magnetic resonance was conducted to evaluate the range and degree of the involvement of the skin, soft tissue, and cranial changes. Although no effective treatment to hold back the progress has been reported so far, surgeries might work to restore the appearance to some extent or improve central nerves symptoms if they exist.

The effects of orchiectomy and steroid on fertility in experimental testicular atrophy.

Despite our understanding of Sertoli cell function and the state of spermatogenesis, the underlying mechanisms remain unclear. This study was conducted to compare the effects of orchiectomy and steroid treatment on fertility in testicular atrophy occurring after testicular torsion. Thirty-three rats were divided into four groups. The atrophy, orchiectomy, and atrophy-steroid groups each contained nine rats, while the control group contained six. The left testes were rotated 720º, and atrophy was observed. In the atrophy-steroid rats, orchiectomy was performed after atrophy, and 1 mg/kg steroid was injected. Each male rat was housed with five female rats for 6 days. The fertility of the male rats was evaluated based on the pregnancy of the female rats. Left and right orchiectomies were performed to determine the tissue Johnsen score (JS) and the serum inhibin B (IB) level. JS values were significantly lower in the atrophy, orchiectomy, and atrophy-steroid groups than in the control group (p<0.05), while no significant difference was observed in JS between the atrophy and orchiectomy groups (p>0.05). Similarly, no significant differences in IB level or fertility percentage were found between the atrophy and orchiectomy rats (p>0.05). In unilateral testicular atrophy, which can occur in the prepubertal period due to various causes, orchiectomy does not appear to benefit fertility, as indicated by IB, JS, and the fertility percentage.

Unilateral pigmented paravenous retinochoroidal atrophy with acute angle-closure glaucoma: a case report

BackgroundPigmented paravenous retinochoroidal atrophy (PPRCA) is an uncommon fundus disease characterized by perivenous aggregations of pigment clumps and retinochoroidal atrophy distributed along the retinal veins. We report a Chinese female case of unilateral PPRCA with acute angle-closure glaucoma (AACG).Case presentationA 50-year-old Chinese female presented with vision loss and elevated intraocular pressure (IOP) in the right eye and then underwent trabeculectomy. She referred to our clinic for further evaluation and treatment. The funduscopic examination revealed grayish retinochoroidal atrophy and osteocyte-like pigment clumping lesions along the retinal veins and peripapillary preretinal hemorrhage in the right eye. The patient also presented with AACG in the same eye on the basis of past medical history of acute attack, shallow anterior chamber depth (ACD), narrow angle showed by ultrasound biomicroscopy (UBM) and glaucomatous neuropathy identified by optical coherence tomography (OCT). Other examinations like fluorescein fundus angiography (FFA), electroretinogram (ERG) and electrooculography (EOG) all confirmed the aforementioned diagnose.ConclusionPPRCA is a rare disease, uncommon in females and symmetrical in both eyes. We present a rare case of unilateral PPRCA accompanied with AACG.

The Presence or Absence of Hippocampal Atrophy Differently Disrupts Graph Theory Properties of Cerebral Functional Connectivity in Temporal Lobe Epilepsy. (S30.004)

<h3>Objective:</h3> To investigate brain connectivity and network properties with graph theory methods in TLE patients according to the presence or absence of HA. <h3>Background:</h3> Although temporal lobe epilepsy (TLE) with unilateral hippocampal atrophy (HA) has been extensively investigated, the impact of bilateral HA and the absence of HA on brain connectivity and networks’ efficiency remains unknown. <h3>Design/Methods:</h3> We analyzed 216 3T-MRI resting-state images of TLE patients (median age: 48 years; 122 women): 92 LEFT-HA, 70 RIGHT-HA, 25 BILATERAL-HA, 29 NEGATIVE-TLE, and 216 healthy controls. We used the CONN connectivity_toolbox/MATLAB2019 to extract graph theory measures (global efficiency (Global-eff), average path length (PATH), degree/cost (DEGREE), betweenness centrality (BC), local efficiency (Local-eff), and clustering coefficient (CC). We considered a cost of 0.15 (including only the 15% more statistically significant results) and reported p-values&lt;0.05. <h3>Results:</h3> The LEFT-HA and RIGHT-HA presented a trend to higher measures of global connectivity (Global-Con), local connectivity (Local-Con), and centrality in the brain (higher Global-eff, DEGREE, BC, Local-eff, CC, and lower PATH) and a trend to lower Global-Con, Local-Con, and centrality in the cerebellum. The LEFT-HA and RIGHT-HA presented higher DEGREE in the ipsilateral hippocampus and higher Local-eff /CC in the contralateral hippocampus. The NEGATIVE-TLE group showed a trend to lower Global-eff and higher average PATH, DEGREE and CC in the brain. The BILATERAL-HA presented a trend to higher Local-eff /CC in the brain. <h3>Conclusions:</h3> Overall, there was a trend for higher local connectivity to the detriment of the Global-eff. Our data indicate more severe alterations in the unilateral groups, as the presence of unilateral HA disturbs hemispheric connectivity, which may disrupt global connections. While the unilateral groups showed higher connectivity levels in the brain (with higher ipsilateral hippocampal Global-Con and probable compensatory higher contralateral hippocampal Local-Con), the NEGATIVE-TLE presented concordantly higher Local-Con and lower Global-Con in the brain. <b>Disclosure:</b> Lucas Silva has nothing to disclose. Dr. Karmann Aventurato has received research support from Coordenadoria de Aperfeiçoamento de Pessoal de Ensino Superior (CAPES). Raphael Casseb has nothing to disclose. The institution of Brunno de Campos has received research support from São Paulo Research Fundation. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB Pharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB Biopharma. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for United Medical – Brazil. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Zodiac Pharma . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Eurofarma – Brazil . Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Epilepsia. Dr. Cendes has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Frontiers in Neurology - Epilepsy. The institution of Dr. Cendes has received research support from São Paulo Research Foundation - FAPESP. The institution of Dr. Cendes has received research support from Conselho Nacional de Desenvolvimento Científico e Tecnológico - Brazil . The institution of Dr. Cendes has received research support from NIH. Clarissa Yasuda has nothing to disclose.

Clinical characteristics and offspring developmental malformation of 126 patients with septate uterus.

Clinical characteristics and offspring developmental malformation of 126 patients with septate uterus.

A case of isolated hypoglossal nerve palsy in a 14-year-old girl. Could Mycoplasma Pneumoniae be the cause?

Hypoglossal Nerve Palsy (HNP) is an uncommon neurological abnormality that can provoke characteristic clinical signs, including unilateral atrophy of the tongue musculature.