-
JB & JS Open Access 2024Acute flaccid myelitis (AFM) is a disabling, poliomyelitis-like illness that mainly affects children. Although various surgical interventions are performed for...
BACKGROUND
Acute flaccid myelitis (AFM) is a disabling, poliomyelitis-like illness that mainly affects children. Although various surgical interventions are performed for intractable paralysis due to AFM, the timing of surgery and its long-term outcomes have yet to be established, especially for shoulder reconstruction. This study aimed to analyze the midterm outcomes of nonsurgically and surgically treated upper-extremity AFM and the factors influencing shoulder functional outcomes after surgical reconstruction.
METHODS
We retrospectively examined 39 patients with AFM in 50 upper extremities between 2011 and 2019. The degree of spontaneous recovery of completely paralyzed muscles was evaluated at a median of 3, 6, and 37 months after the onset of paralysis. Twenty-seven patients with 29 extremities underwent surgery involving nerve transfer, muscle-tendon transfer, or free muscle transfer for shoulder, elbow, and hand reconstruction.
RESULTS
Patients with complete paralysis of shoulder abduction at 6 months did not show later recovery. Twenty-two patients with 24 extremities underwent shoulder surgery, and all but 1 were followed for at least 24 months after surgery. Although postoperative shoulder abduction recovery was similar between transfer of the spinal accessory nerve and of the contralateral C7 nerve root to the suprascapular nerve, the outcomes obtained with spinal accessory nerve transfer had more variability, likely related to latent spinal accessory nerve paralysis, shoulder instability related to pectoralis major paralysis, and the type of paralysis. Shoulder abduction recovery was also greatly affected by scapulothoracic joint movement. In contrast, the outcomes of the elbow flexion and hand reconstructions were more consistent and acceptable.
CONCLUSIONS
All patients had loss of shoulder abduction, and restoration of shoulder function was less predictable and depended on the quality of the donor nerves and recovery of the synergistic muscles. Strict donor nerve selection and additional nerve transfer for shoulder reconstruction are imperative for satisfactory outcomes.
LEVEL OF EVIDENCE
Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.
PubMed: 38774108
DOI: 10.2106/JBJS.OA.23.00143 -
Frontiers in Neurology 2024Multiple sclerosis (MS) is a demyelinating disorder of the central nervous system. Increasing evidence indicates additional peripheral nerve involvement in early and...
OBJECTIVES
Multiple sclerosis (MS) is a demyelinating disorder of the central nervous system. Increasing evidence indicates additional peripheral nerve involvement in early and chronic disease stages. To investigate the evolution of peripheral nerve changes in patients first diagnosed with MS using quantitative MR neurography.
MATERIALS AND METHODS
This prospective study included 19 patients with newly diagnosed MS according to the revised McDonald criteria (16 female, mean 30.2 ± 7.1 years) and 19 age-/sex-matched healthy volunteers. High-resolution 3 T MR neurography of the sciatic nerve using a quantitative T2-relaxometry sequence was performed, which yielded the biomarkers of T2 relaxation time (T2app) and proton spin density (PSD). Follow-up scans of patients were performed after median of 12 months (range 7-16). Correlation analyses considered clinical symptoms, intrathecal immunoglobulin synthesis, nerve conduction study, and lesion load on brain and spine MRI.
RESULTS
Patients showed increased T2app and decreased PSD compared to healthy controls at initial diagnosis and follow-up ( < 0.001 each). Compared to the initial scan, T2app further increased in patients at follow-up ( = 0.003). PSD further declined by at least 10% in 9/19 patients and remained stable in another 9/19 patients. Correlation analyses did not yield significant results.
CONCLUSION
Peripheral nerve involvement in MS appears at initial diagnosis and continues to evolve within 1 year follow-up with individual dynamics. Quantitative MRN provides non-invasive biomarkers to detect and monitor peripheral nerve changes in MS.
PubMed: 38765263
DOI: 10.3389/fneur.2024.1335408 -
Scientific Reports May 2024The present study examined the effects of transcutaneous auricular vagus nerve stimulation (taVNS) on short-latency afferent inhibition (SAI), as indirect biomarker of...
The present study examined the effects of transcutaneous auricular vagus nerve stimulation (taVNS) on short-latency afferent inhibition (SAI), as indirect biomarker of cholinergic system activation. 24 healthy adults underwent intermittent taVNS (30 s on/30 s off, 30 min) or continuous taVNS at a frequency of 25 Hz (15 min) along with earlobe temporary stimulation (15 min or 30 min) were performed in random order. The efficiency with which the motor evoked potential from the abductor pollicis brevis muscle by transcranial magnetic stimulation was attenuated by the preceding median nerve conditioning stimulus was compared before taVNS, immediately after taVNS, and 15 min after taVNS. Continuous taVNS significantly increased SAI at 15 min post-stimulation compared to baseline. A positive correlation (Pearson coefficient = 0.563, p = 0.004) was observed between baseline SAI and changes after continuous taVNS. These results suggest that 15 min of continuous taVNS increases the activity of the cholinergic nervous system, as evidenced by the increase in SAI. In particular, the increase after taVNS was more pronounced in those with lower initial SAI. This study provides fundamental insight into the clinical potential of taVNS for cholinergic dysfunction.
Topics: Humans; Male; Female; Adult; Vagus Nerve Stimulation; Transcutaneous Electric Nerve Stimulation; Young Adult; Evoked Potentials, Motor; Transcranial Magnetic Stimulation; Vagus Nerve
PubMed: 38755234
DOI: 10.1038/s41598-024-61958-8 -
NeuroImage. Clinical 2024Previous studies have raised concerns regarding neurodevelopmental impacts of early exposures to general anesthesia and surgery. Electroencephalography (EEG) can be used...
BACKGROUND
Previous studies have raised concerns regarding neurodevelopmental impacts of early exposures to general anesthesia and surgery. Electroencephalography (EEG) can be used to study ontogeny of brain networks during infancy. As a substudy of an ongoing study, we examined measures of functional connectivity in awake infants with prior early and prolonged anesthetic exposures and in control infants.
METHODS
EEG functional connectivity was assessed using debiased weighted phase lag index at source and sensor levels and graph theoretical measures for resting state activity in awake infants in the early anesthesia (n = 26 at 10 month visit, median duration of anesthesia = 4 [2, 7 h]) and control (n = 38 at 10 month visit) groups at ages approximately 2, 4 and 10 months. Theta and low alpha frequency bands were of primary interest. Linear mixed models incorporated impact of age and cumulative hours of general anesthesia exposure.
RESULTS
Models showed no significant impact of cumulative hours of general anesthesia exposure on debiased weighted phase lag index, characteristic path length, clustering coefficient or small-worldness (conditional R 0.05-0.34). An effect of age was apparent in many of these measures.
CONCLUSIONS
We could not demonstrate significant impact of general anesthesia in the first months of life on early development of resting state brain networks over the first postnatal year. Future studies will explore these networks as these infants grow older.
Topics: Humans; Infant; Male; Female; Brain; Electroencephalography; Anesthesia, General; Nerve Net; Child Development
PubMed: 38754325
DOI: 10.1016/j.nicl.2024.103614 -
Plastic and Reconstructive Surgery.... May 2024Carpal tunnel syndrome (CTS) is a clinical diagnosis involving numerous confirmatory diagnostic tools, including patient questionnaires, ultrasound (US), and...
BACKGROUND
Carpal tunnel syndrome (CTS) is a clinical diagnosis involving numerous confirmatory diagnostic tools, including patient questionnaires, ultrasound (US), and electrodiagnostic studies (EDX.) Patients may experience clinical symptoms of CTS with false negative diagnostic testing. The purpose of this study was to identify characteristics of patients with clinical symptoms of CTS with negative diagnostic testing.
METHODS
An existing database of 295 hands containing the six-item CTS-6, US of the median nerve, and EDX was queried. Patients with symptoms of carpal tunnel scoring 12.5 or higher on CTS-6 were sorted into those with all positive testing or negative testing.
RESULTS
In 60 patients, 103 hands had both positive US and EDX and a CTS-6 of 12.5 or higher. Twenty-nine hands in 25 patients had a CTS-6 of 12.5 or higher and both negative ultrasound and EDX. There was a significantly younger average age of 43 ( = 0.007) and lower average BMI of 28 ( < 0.0001) of patients in the negative diagnostic study group, compared with the average age of 53, and a body mass index (BMI) of 34 in the positive diagnostic study.
CONCLUSIONS
In this series, patients with symptoms of carpal tunnel syndrome and negative diagnostic studies were on average younger and had a lower BMI. These patients may warrant more careful consideration of CTS clinical diagnosis and counseling regarding a higher risk of false-negative confirmatory testing. Further studies are needed to determine possible effects of age and BMI on electrodiagnostic studies and ultrasound testing in CTS.
PubMed: 38752222
DOI: 10.1097/GOX.0000000000005816 -
Annals of Indian Academy of Neurology 2024Friedreich's ataxia (FRDA) is a common cause of autosomal recessive cerebellar ataxia. The phenotype is dependent on the repeat size and duration of the disease. We...
BACKGROUND AND AIM
Friedreich's ataxia (FRDA) is a common cause of autosomal recessive cerebellar ataxia. The phenotype is dependent on the repeat size and duration of the disease. We aimed to study the clinical, electrophysiologic, and radiologic profiles in a large Indian cohort of genetically proven FRDA patients.
SUBJECTS AND METHODS
A retrospective cross-sectional, descriptive analysis of genetically proven FRDA patients was performed. A detailed review of all the hospital case records was done to analyze the clinical, radiologic, and electrophysiologic details.
RESULTS
A total of 100 FRDA patients were selected for the analysis. Eighty-six patients had an age at onset between 5 and 25 years. Eight patients (8%) were classified as late-onset FRDA and six patients (6%) as early-onset FRDA. The median age at presentation was 19 years. The median age at onset was 14 years, and the median duration of illness was 4 years. All patients had gait ataxia as the initial symptom. Gait ataxia, loss of proprioception, and areflexia were seen in all patients. Dysarthria, nystagmus, amyotrophy, spasticity, extensor plantars, pes cavus, and scoliosis occurred in one-third of patients. Cardiomyopathy (18%) and diabetes (5%) were less common. Sensory polyneuropathy (87.5%) was the most common nerve conduction abnormality. Cortical somatosensory evoked responses were absent in all 43 tested patients (100%). Brainstem auditory evoked response test was done in 24 patients and it showed absent reactions in six patients (25%). Visual evoked potential was tested in 24 patients and it showed absent P100 responses in five patients (21%). Cerebellar and cord atrophy was seen on magnetic resonance imaging in 50% of patients.
CONCLUSION
Most FRDA patients (86%) had an age at onset of less than 25 years, with typical symptoms of gait ataxia, areflexia, and loss of proprioception found in all patients. Dysarthria, nystagmus, amyotrophy, spasticity, extensor plantars, pes cavus, scoliosis, cardiomyopathy, and diabetes were not seen in all patients. Cerebellar atrophy can occur in FRDA patients. Knowledge regarding the clinical, radiologic, and electrophysiologic profile of FRDA will aid in proper phenotypic characterization.
PubMed: 38751907
DOI: 10.4103/aian.aian_1001_23 -
Case Reports in Plastic Surgery & Hand... 2024Carpal tunnel syndrome is the most common entrapment neuropathy in the upper extremity. Palmaris longus, flexor digitorum superficialis, and lumbricals have infrequently...
Carpal tunnel syndrome is the most common entrapment neuropathy in the upper extremity. Palmaris longus, flexor digitorum superficialis, and lumbricals have infrequently been reported as causes of nerve compression. During routine Korean cadaver dissection, we incidentally identified an anatomic variant of first lumbrical muscle within the carpal tunnel in both wrists. The aberrant musculature originated from the radial side of the second FDS muscle at distal forearm level, running separately across the wrist beneath the flexor retinaculum. The dissected anomalous muscle was identified as an additional muscle belly of the first lumbrical muscle. Compression of the median nerve at the wrist might rarely be caused by the presence of such a tendon or muscle anomaly found in this study. Surgeons should be aware of possible anatomic variations in the carpal tunnel, and be prepared to modify their surgical plan accordingly.
PubMed: 38751547
DOI: 10.1080/23320885.2024.2351130 -
Ocular Oncology and Pathology Apr 2024The objective of this study was to report the clinicopathologic features of three cases of -amplified retinoblastoma identified genetically by aqueous humor sampling.
INTRODUCTION
The objective of this study was to report the clinicopathologic features of three cases of -amplified retinoblastoma identified genetically by aqueous humor sampling.
METHODS
Whole-genome sequencing was performed using isolated cell-free DNA (cfDNA) from aqueous humor of 3 retinoblastoma patients. We analyzed genomic copy number and mutational alterations, histologic and pathologic features, and clinical data.
RESULTS
The most common genetic alteration identified in these three retinoblastoma cases was a focal amplification on 2p. All tumors showed an early age of diagnosis with a median of 9 months. The tumor histopathologic features included neovascularization and subretinal seeding in case 1, diffuse nature with choroidal and prelaminar optic nerve invasion in case 2, and complete vitreous seeding in case 3. Case 1 expressed RB protein and had no mutation, case 2 did not express RB protein and had an mutation, and case 3 did not express RB protein and likely had an epigenetic effect on RB expression.
CONCLUSIONS
Our report shows 3 cases of unilateral retinoblastomas diagnosed in patients ranging from 4 months to 18 months old. Genomic analysis from AH cfDNA revealed amplification with intact RB protein staining in case 1 and lack of RB staining in cases 2 and 3. mutational analysis in the AH confirmed a pathogenic variant in case 2. Clinical pathology showed features requiring aggressive treatment, specifically enucleation.
IMPORTANCE
-amplified retinoblastomas demonstrate unique pathogenesis and aggressive behavior, regardless if is a primary or secondary driver of disease. Genomic analysis from aqueous humor may be useful when deciding to enucleate as opposed to treating conservatively. Focal amplification on 2p might be relevant for tumor growth in this subset of the retinoblastoma population in terms of targeted therapeutics.
PubMed: 38751495
DOI: 10.1159/000533311 -
JPMA. the Journal of the Pakistan... Apr 2024Soft tissue swellings on the forearm can present with a range of clinical and histopathological diagnosis. Ancient Schawanoma is a rare benign condition that can develop...
Soft tissue swellings on the forearm can present with a range of clinical and histopathological diagnosis. Ancient Schawanoma is a rare benign condition that can develop over the flexor surface of the forearm as a cystic swelling and can involve the median or the ulnar nerve. However, the presentation of this condition on the extensor surface with involvement of the radial nerve is an extremely uncommon diagnosis. A 69 year old female presented at the outpatient department with a swelling on the extensor aspect of her right forearm for the past 2 years. Ultrasound examination showed a mixed cystic solid mass and MRI report revealed a complex predominantly cystic mass in the extensor compartment of the forearm, measuring 4.3 x 5.3 x 7.2 cm size. After obtaining informed consent, the patient was operated under tourniquet control and the mass was removed sparing the radial nerve that was adherent to its capsule. The final histopathological report confirmed the diagnosis as Ancient Schawanoma.
Topics: Humans; Female; Aged; Radial Nerve; Magnetic Resonance Imaging; Radial Neuropathy; Forearm; Ultrasonography
PubMed: 38751285
DOI: 10.47391/JPMA.9610 -
CNS Neuroscience & Therapeutics May 2024With the improvement of emergency techniques, the survival rate of patients with severe brain injury has increased. However, this has also led to an annual increase in... (Review)
Review
BACKGROUND
With the improvement of emergency techniques, the survival rate of patients with severe brain injury has increased. However, this has also led to an annual increase in the number of patients with prolonged disorders of consciousness (pDoC). Hence, recovery of consciousness is an important part of treatment. With advancing techniques, noninvasive neuromodulation seems a promising intervention. The objective of this review was to summarize the latest techniques and provide the basis for protocols of noninvasive neuromodulations in pDoC.
METHODS
This review summarized the advances in noninvasive neuromodulation in the treatment of pDoC in the last 5 years.
RESULTS
Variable techniques of neuromodulation are used in pDoC. Transcranial ultrasonic stimulation (TUS) and transcutaneous auricular vagus nerve stimulation (taVNS) are very new techniques, while transcranial direct current stimulation (tDCS) and transcranial magnetic stimulation (TMS) are still the hotspots in pDoC. Median nerve electrical stimulation (MNS) has received little attention in the last 5 years.
CONCLUSIONS
Noninvasive neuromodulation is a valuable and promising technique to treat pDoC. Further studies are needed to determine a unified stimulus protocol to achieve optimal effects as well as safety.
Topics: Humans; Consciousness Disorders; Transcranial Magnetic Stimulation; Transcranial Direct Current Stimulation; Vagus Nerve Stimulation; Transcutaneous Electric Nerve Stimulation
PubMed: 38747078
DOI: 10.1111/cns.14757