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Neurologia I Neurochirurgia Polska Jun 2024Lambert-Eaton myasthenic syndrome (LEMS) is an ultrarare neuromuscular disease with a triad of symptoms: muscle paresis, dysautonomy, and areflexia. Amifampridine is the...
INTRODUCTION
Lambert-Eaton myasthenic syndrome (LEMS) is an ultrarare neuromuscular disease with a triad of symptoms: muscle paresis, dysautonomy, and areflexia. Amifampridine is the symptomatic treatment of LEMS.
AIM OF STUDY
To assess the effectiveness and safety of treatment in the real world.
MATERIAL AND METHODS
14 patients with non-neoplastic LEMS treated with amifampridine were enrolled in the study (female 42.9%, mean age 48.8 ± 11.4 years). The patients were assessed using the Quantitative Myasthenia Gravis (QMG) scale, QMG limb domain (LD) score, spirometry, Hand Grip Strength (GRIP) test, and repetitive nerve stimulation study (RNS) at baseline and at the end of follow-up. Diagnostic delay since first symptoms was from seven months up to 22 years. Treatment delay ranged from one to 26 years. The patients were treated and reevaluated after 21.1 ± 12.0 weeks (range 13-48).
RESULTS
All of the patients improved in QMG score. Mean improvement was 5.1 ± 2.0 (range 1-8) points (p < 0.001) and this showed no correlation with the duration of the disease before treatment (p = 0.477). 85.7% of patients (N = 12) improved ≥ 3 points (clinically meaningful) in QMG. 78.6% of the patients improved in QMG LD (mean 2.2 ± 1.6 points (p < 0.001)). Also, forced vital capacity (FVC) improved after treatment (p = 0.031). Mean improvement in GRIP test was 7.0 ± 7.1 kg in the right hand and 5.2 ± 7.5 kg in the left hand (p < 0.001). In RNS before treatment, facilitation ( > 100%) was observed in 78.6% (N = 11) of patients, and was higher before treatment (p < 0.001). Compound muscle action potential (CMAP) amplitude was higher after treatment (p < 0.001). Mean increase of CMAP amplitude was 2.1 ± 1.6 times. In 64.3% (N = 9) of patients lowering of corticosteroid dose was achieved.
CONCLUSIONS
Amifampridine is an effective treatment in non-neoplastic LEMS patients, regardless of disease duration. The treatment is well-tolerated and allows to reduce dose of corticosteroids in the majority of patients.
PubMed: 38935421
DOI: 10.5603/pjnns.99335 -
Sensors (Basel, Switzerland) Jun 2024After a stroke, antagonist muscle activation during agonist command impedes movement. This study compared measurements of antagonist muscle activation using surface...
After a stroke, antagonist muscle activation during agonist command impedes movement. This study compared measurements of antagonist muscle activation using surface bipolar EMG in the gastrocnemius medialis (GM) and high-density (HD) EMG in the GM and soleus (SO) during isometric submaximal and maximal dorsiflexion efforts, with knee flexed and extended, in 12 subjects with chronic hemiparesis. The coefficients of antagonist activation (CAN) of GM and SO were calculated according to the ratio of the RMS amplitude during dorsiflexion effort to the maximal agonist effort for the same muscle. Bipolar CAN (BipCAN) was compared to CAN from channel-specific (CsCAN) and overall (OvCAN) normalizations of HD-EMG. The location of the CAN centroid was explored in GM, and CAN was compared between the medial and lateral portions of SO. Between-EMG system differences in GM were observed in maximal efforts only, between BipCAN and CsCAN with lower values in BipCAN ( < 0.001), and between BipCAN and OvCAN with lower values in OvCAN ( < 0.05). The CAN centroid is located mid-height and medially in GM, while the CAN was similar in medial and lateral SO. In chronic hemiparesis, the estimates of GM hyperactivity differ between bipolar and HD-EMGs, with channel-specific and overall normalizations yielding, respectively, higher and lower CAN values than bipolar EMG. HD-EMG would be the way to develop personalized rehabilitation programs based on individual antagonist activations.
Topics: Humans; Electromyography; Paresis; Male; Female; Muscle, Skeletal; Middle Aged; Aged; Adult; Chronic Disease; Stroke
PubMed: 38931485
DOI: 10.3390/s24123701 -
Medicina (Kaunas, Lithuania) Jun 2024: Microsurgical resection with intraoperative neuromonitoring is the gold standard for acoustic neurinomas (ANs) which are classified as T3 or T4 tumors according to the...
: Microsurgical resection with intraoperative neuromonitoring is the gold standard for acoustic neurinomas (ANs) which are classified as T3 or T4 tumors according to the Hannover Classification. Microscope-based augmented reality (AR) can be beneficial in cerebellopontine angle and lateral skull base surgery, since these are small areas packed with anatomical structures and the use of this technology enables automatic 3D building of a model without the need for a surgeon to mentally perform this task of transferring 2D images seen on the microscope into imaginary 3D images, which then reduces the possibility of error and provides better orientation in the operative field. : All patients who underwent surgery for resection of ANs in our department were included in this study. Clinical outcomes in terms of postoperative neurological deficits and complications were evaluated, as well as neuroradiological outcomes for tumor remnants and recurrence. : A total of 43 consecutive patients (25 female, median age 60.5 ± 16 years) who underwent resection of ANs via retrosigmoid osteoclastic craniotomy with the use of intraoperative neuromonitoring (22 right-sided, 14 giant tumors, 10 cystic, 7 with hydrocephalus) by a single surgeon were included in this study, with a median follow up of 41.2 ± 32.2 months. A total of 18 patients underwent subtotal resection, 1 patient partial resection and 24 patients gross total resection. A total of 27 patients underwent resection in sitting position and the rest in semi-sitting position. Out of 37 patients who had no facial nerve deficit prior to surgery, 19 patients were intact following surgery, 7 patients had House Brackmann (HB) Grade II paresis, 3 patients HB III, 7 patients HB IV and 1 patient HB V. Wound healing deficit with cerebrospinal fluid (CSF) leak occurred in 8 patients (18.6%). Operative time was 317.3 ± 99 min. One patient which had recurrence and one further patient with partial resection underwent radiotherapy following surgery. A total of 16 patients (37.2%) underwent resection using fiducial-based navigation and microscope-based AR, all in sitting position. Segmented objects of interest in AR were the sigmoid and transverse sinus, tumor outline, cranial nerves (CN) VII, VIII and V, petrous vein, cochlea and semicircular canals and brain stem. Operative time and clinical outcome did not differ between the AR and the non-AR group. However, use of AR improved orientation in the operative field for craniotomy planning and microsurgical resection by identification of important neurovascular structures. : The single-center experience of resection of ANs showed a high rate of gross total (GTR) and subtotal resection (STR) with low recurrence. Use of AR improves intraoperative orientation and facilitates craniotomy planning and AN resection through early improved identification of important anatomical relations to structures of the inner auditory canal, venous sinuses, petrous vein, brain stem and the course of cranial nerves.
Topics: Humans; Female; Middle Aged; Male; Microsurgery; Neuroma, Acoustic; Aged; Adult; Augmented Reality; Neurosurgical Procedures; Microscopy; Treatment Outcome; Imaging, Three-Dimensional
PubMed: 38929549
DOI: 10.3390/medicina60060932 -
Animals : An Open Access Journal From... Jun 2024Nutritional secondary hyperparathyroidism (NSH) in dogs is a condition that develops in response to a vitamin D deficiency or an imbalanced calcium-to-phosphorus ratio...
Nutritional secondary hyperparathyroidism (NSH) in dogs is a condition that develops in response to a vitamin D deficiency or an imbalanced calcium-to-phosphorus ratio in dog food. Puppies of large-breed dogs exclusively fed a non-supplemented, boneless raw meat diet are especially susceptible to developing NSH due to their elevated calcium requirement. Reports on NSH in companion animals have been sparse in the last decades due to dog owners having easy access to commercially balanced dog foods. However, with the rising popularity of meat-based raw feeding, this condition has re-emerged. In this case series, four large-breed puppies fed exclusively non-supplemented, boneless raw meat diets presented with complaints of acute onset of pain and paresis. Radiographs and/or computed tomography (CT) scans showed reduced radio density of the skeleton in all four puppies. Two of the dogs had pathological fractures, and these two puppies were euthanized. One was subjected to a post mortem examination, which revealed cortical bone resorption and hypertrophy of the parathyroid glands. The remaining two puppies rapidly improved after receiving pain medication and a commercial, balanced diet. This case series demonstrates a risk of young dogs developing severe neurological deficits when fed a non-supplemented, boneless raw meat diet.
PubMed: 38929402
DOI: 10.3390/ani14121783 -
Diagnostics (Basel, Switzerland) Jun 2024In 2019, mild vestibular function deficiency in elder populations was defined as presbyvestibulopathy (PVP) by the Classification Committee of the Bárány Society. The...
INTRODUCTION
In 2019, mild vestibular function deficiency in elder populations was defined as presbyvestibulopathy (PVP) by the Classification Committee of the Bárány Society. The diagnostic criteria include tests for low-, mid-, and high-frequency vestibular function, represented by caloric testing, rotary chair testing, and head impulse testing, respectively. However, there is still a lack of large-scale reports supporting the relationship between vestibular function tests (VFTs) and aging. In this study, we evaluated whether each test is correlated with aging in the elderly population aged over 50.
METHODS
This study retrospectively enrolled 1043 subjects from a single university hospital database after excluding those with unilateral and bilateral vestibulopathy, central dizziness, and acute dizziness. Enrolled subjects had caloric canal paresis <20%, vHIT lateral canal gain >0.6, vHIT interaural difference <0.3, and age >50 years old.
RESULTS
Significant negative correlations with age were identified in the vHIT ( < 0.001) and rotary chair test (RCT) 1.0 Hz gain ( = 0.030). However, the caloric test ( = 0.739 and 0.745 on the left and right sides, respectively) and RCT 0.12 Hz gain ( = 0.298) did not show a significant correlation with age. A total of 4.83% of subjects aged 70 years or older showed sub-normal vHIT gain that met the criteria of PVP, whereas only 0.50% of subjects aged 60 to 69 did. The prevalence of sub-normal caloric test results, however, was not significantly different between the two age groups (21.55% in the 60-69 age group and 26.59% in the >70 age group).
CONCLUSIONS
The high-frequency range vestibular function seems vulnerable to aging, and this is more discernible at age >70 years. The weak correlation between age and low-frequency vestibular function tests, such as the caloric test and low-frequency rotary chair testing, suggests the need to revisit the diagnostic criteria for PVP.
PubMed: 38928640
DOI: 10.3390/diagnostics14121224 -
Toxins May 2024This retrospective, observational study describes the clinical findings, case management trends, and outcomes of 83 dogs and nine cats exposed to eastern coral snakes in...
Retrospective Evaluation of Clinical and Clinicopathologic Findings, Case Management, and Outcome for Dogs and Cats Exposed to (Eastern Coral Snake): 92 Cases (2021-2022).
This retrospective, observational study describes the clinical findings, case management trends, and outcomes of 83 dogs and nine cats exposed to eastern coral snakes in a university teaching hospital setting. The medical records of dogs and cats that received antivenom following coral snake exposure were reviewed. Data collected included signalment, time to antivenom administration, physical and laboratory characteristics at presentation, clinical course during hospitalization, length of hospitalization, and survival to discharge. The mean time from presentation to coral snake antivenom administration was 2.26 ± 1.46 h. Excluding cases where the owner declined in-hospital care, the mean hospitalization time for dogs and cats was 50.8 h and 34 h, respectively. The mean number of antivenom vials was 1.29 (1-4). Gastrointestinal signs (vomiting and ptyalism) occurred in 42.2% (35/83) of dogs and 33.3% (3/9) of cats. Peripheral neurologic system deficits (ataxia, paresis to plegia, absent reflexes, and hypoventilation) were noted in 19.6% (18/92) of dogs and cats. Hemolysis was also common in 37.9% (25/66) of dogs but was not observed in cats. Mechanical ventilation (MV) was indicated in 12% (10/83) of dogs but no cats. Acute kidney injury (AKI), while rare, was a common cause of euthanasia at 20% (2/5) and was the most common complication during MV at 44.4% (4/9). Pigmenturia/hemolysis occurred in 88.9% (8/9) of MV cases and in all cases with AKI. Despite delays in antivenom administration by several hours, dogs and cats with coral snake exposure have low mortality rates (6% of dogs (5/83) and 0% of cats). Gastrointestinal signs were common but were not predictive of progression to neurological signs. Thus, differentiating between coral snake exposure and envenomation before the onset of neurological signs remains challenging.
Topics: Animals; Dogs; Antivenins; Retrospective Studies; Cats; Coral Snakes; Snake Bites; Cat Diseases; Elapid Venoms; Male; Female; Dog Diseases; Treatment Outcome; Venomous Snakes
PubMed: 38922141
DOI: 10.3390/toxins16060246 -
Journal of Orthopaedic Case Reports Jun 2024The results of primary total knee replacement (TKR) using hinge implants performed in the Indian population with post-polio residual paresis (PPRP) are unknown. The...
INTRODUCTION
The results of primary total knee replacement (TKR) using hinge implants performed in the Indian population with post-polio residual paresis (PPRP) are unknown. The purpose of this study was to report the outcome of primary rotating hinge TKR in Indian patients with PPRP at a minimum follow-up of 12 months.
MATERIALS AND METHODS
We retrospectively reviewed the clinical and radiological records of six patients treated with primary rotating hinge TKR. Pre-and post-operative (at final follow-up) knee range of motion (ROM), knee sagittal deformity, knee society score (KSS), and Oxford knee score (OKS) were compared to determine improvement in function.
RESULTS
Six rotating hinge TKRs (five female and one male patient) were analyzed for this study. At a mean follow-up of 27 ± 22 months (range, 12-71 months), the mean pre-operative KSS of 50.6 ± 2.5 significantly improved (P < 0.0001) to 72.5 ± 1.6, and the mean pre-operative OKS of 23.6 ± 1.6 significantly improved (P < 0.0001) to 35.3 ± 1.7. The mean pre-operative knee ROM of 94° ± 10° changed to 92° ± 4° (P = 0.64) and the mean pre-operative sagittal deformity of 7° ± 23.5° changed to -3° ± 2.5° (P = 0.32) at final follow-up. None of the knees had any intra- or post-operative complications or showed radiologic evidence of post-operative loosening, subsidence, or periprosthetic radiolucent lines at the final follow-up.
CONCLUSION
Rotating hinge TKR gave excellent clinical and radiological results at a mean follow-up of 27 months in the present study. Despite TKR being a technically challenging procedure in patients with poliomyelitis-affected limbs, a rotating hinge design, along with meticulous surgical technique, can significantly improve function in such patients.
PubMed: 38910982
DOI: 10.13107/jocr.2024.v14.i06.4542 -
Journal of Cardiothoracic Surgery Jun 2024This manuscript aims to describe the symptoms, demographics, surgical approaches and techniques, the volume of surgical interventions, histological results, intra- and...
BACKGROUND
This manuscript aims to describe the symptoms, demographics, surgical approaches and techniques, the volume of surgical interventions, histological results, intra- and postoperative complications, and postoperative results in patients with anterior mediastinal tumors of thyroid origin (AMTTO).
METHODS
Twenty patients with AMTTO were operated between 2017 and 2021. Fifteen were women and 5 were men. The mean age was 66.8 years.
RESULTS
The most common histology was nodular micro- and macrofollicular goiter (15/20, 75%). Kocher cervicotomy (65%) was the preferred approach. Total thyroidectomy was performed in 95% of patients. Intraoperative complications were identified in 25% (5/20), and in 2 patients a tracheostomy was required. Early postoperative complications were established in 65% and the most common was unilateral transient recurrent nerve paresis or paralysis and dysphonia (25%).
CONCLUSIONS
Commonly resection of AMTTO is a challenge due to its complexities associated with high-risk cases, emphasizing the need for experienced centers in managing such cases.
Topics: Humans; Male; Female; Aged; Mediastinal Neoplasms; Thyroidectomy; Middle Aged; Thyroid Neoplasms; Retrospective Studies; Postoperative Complications; Treatment Outcome; Adult; Intraoperative Complications; Thyroid Gland; Aged, 80 and over
PubMed: 38907269
DOI: 10.1186/s13019-024-02831-7 -
Sexually Transmitted Infections Jun 2024This study aimed to describe the clinical features of neurosyphilis in Chinese patients in an attempt to find clinical features that are helpful for the early...
OBJECTIVES
This study aimed to describe the clinical features of neurosyphilis in Chinese patients in an attempt to find clinical features that are helpful for the early identification of neurosyphilis.
METHODS
This retrospective study included people with syphilis who visited Shanghai Skin Disease Hospital between January 2010 and December 2020. Lumbar puncture was performed on those who met the inclusion and exclusion criteria. The diagnosis of neurosyphilis was based on clinical and laboratory findings. The parameters were analysed statistically.
RESULTS
Of the 3524 patients with neurosyphilis, 2111 (59.9%) and 1413 (40.1%) were asymptomatic and symptomatic neurosyphilis, respectively. General paresis was the most common type of symptomatic neurosyphilis (46.8%). The clinical manifestations of symptomatic neurosyphilis include psychiatric and neurotic symptoms, among which general paresis predominantly presented as psychiatric symptoms such as affective (66.7%) and memory disorder (72.9%). Tabes dorsalis is often presented as neurotic symptoms. One hundred fifty patients (10.6%) with symptomatic neurosyphilis presented candy signs, a rare and specific neurosyphilis symptom that is common in general paresis. Girdle sensation was presented in 13 patients, mainly with tabes dorsalis, which had not been reported in previous studies.
CONCLUSIONS
Notably, the candy sign is identified as a specific symptom of general paresis, while girdle sensations are highlighted as a particular symptom of tabes dorsalis. This is the largest study describing the clinical spectrum of neurosyphilis since the onset of the penicillin era and could help doctors learn more about the disease. A comprehensive description of the possible clinical manifestations of late symptomatic neurosyphilis, particularly highlighting rare symptoms, can identify suspicious patients and prevent diagnostic delays.
PubMed: 38902025
DOI: 10.1136/sextrans-2024-056117 -
Journal of Medical Case Reports Jun 2024Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This...
BACKGROUND
Hepatic myelopathy is a very rare neurological complication of chronic liver disease. Patients habitually present with progressive pure motor spastic paraparesis. This neurological dysfunction is almost always due to cirrhosis and portocaval shunt, either surgical or spontaneous.
CASES REPORT
We report two cases of a 57-year-old man and a 37-year-old woman with progressive spastic paraparesis linked to cirrhosis and portal hypertension. The two patients are of Tunisian origin (north Africa). Magnetic resonance imaging of the spinal cord of two patients was normal, while brain magnetic resonance imaging showed a T2 hypersignals of the pallidums. These signs, in favor of hepatic encephalopathy in the two patients with cirrhosis with isolated progressive spastic paraparesis without bladder or sensory disorders, help to retain the diagnosis of hepatic myelopathy.
CONCLUSION
Hepatic myelopathy is a severe and debilitating neurological complication of chronic liver disease. The pathogenesis is misunderstood and seems to be multifactorial, including the selective neurotoxic role both of ammonia and other pathogenic neurotoxins. Usually a pathological brain magnetic resonance imaging showing a hepatic encephalopathy was documented, contrasting with a normal spinal cord magnetic resonance imaging that contributed to diagnosis of hepatic myelopathy. Conservative therapies such as ammonia-lowering measures, diet supplementation, antispastic drugs, and endovascular shunt occlusion show little benefit in improving disease symptoms. Liver transplantation performed at early stage can prevent disease progression and could probably allow for recovery.
Topics: Humans; Female; Middle Aged; Male; Hepatic Encephalopathy; Adult; Magnetic Resonance Imaging; Spinal Cord Diseases; Liver Cirrhosis; Paraparesis, Spastic; Hypertension, Portal; Chronic Disease
PubMed: 38880918
DOI: 10.1186/s13256-024-04495-2