Lacertus syndrome: recent advances.

Lacertus syndrome consists in proximal median nerve entrapment with median nerve compression at the lacertus fibrosus, causing hand weakness and fatigue, forearm pain and occasional numbness. Recent advances emphasized the importance of clinical examination, due to limitations in electromyographic diagnosis and delayed diagnosis. The Hagert clinical triad, lacertus notch sign, lacertus antagonist test and taping help accurate diagnosis. Non-operative treatment should be tried; and surgical techniques, whether open or ultrasound-guided under WALANT (wide-awake, local anesthesia, no tourniquet) show promising outcomes. Improved awareness, accurate diagnosis and innovative treatments enhance patient care for this challenging condition.

Relationships between the global symptom score and electrophysiological findings after surgical release for carpal tunnel syndrome: Indications and outcomes.

PURPOSE: Regarding surgical indications for carpal tunnel syndrome (CTS), the hypothesis that the recovery processes of subjective symptoms differ among pain, sensory, and motor symptoms and correlate with recovery in objective nerve conduction studies was examined in the present study. METHODS: The global symptom score (GSS) is a method used to assess clinical outcomes and covers subjective symptoms, including pain (pain and nocturnal awakening), sensory (numbness and paresthesia), and motor (weakness/clumsiness) symptoms. The relationships between long-term changes in GSS and recovery in nerve conduction studies were investigated. RESULTS: Forty patients (40 hands) were included (mean age 65 years; 80% female; 68% with moderate CTS: sensory nerve conduction velocity < 45 m/s and motor nerve distal latency > 4.5 ms). Pain and nocturnal awakening rapidly subsided within 1 month after surgery and did not recur in the long term (median 5.6 years). Paresthesia significantly decreased 3 months after surgery and in the long term thereafter. Weakness/clumsiness significantly decreased at 1 year. Sensory nerve distal latency, conduction velocity, and amplitude significantly improved 3 months and 1 year after surgery, and correlated with nocturnal awakening in the short term (3 months) in moderate CTS cases. The patient satisfaction rate was 91%. CONCLUSION: Rapid recovery was observed in pain and nocturnal awakening, of which nocturnal awakening correlated with the recovery of sensory nerve conduction velocity. Patients with pain symptoms due to moderate CTS may benefit from surgical release.

Exploring pathogenic pathways in carpal tunnel syndrome: sterile inflammation and oxidative stress.

OBJECTIVES: The main objective of the current study was to find the association between oxidative stress, inflammatory markers, and electrophysiological profile with symptom severity in patients of carpal tunnel syndrome (CTS). METHODS: Thirty-two carpal tunnel syndrome patients and 32 controls were included in the study. Boston CTS questionnaire along with plasma oxidative stress markers including superoxide dismutase, malondialdehyde, and nitric oxide and inflammatory markers including IL-6 and TNF-α were compared with the electrophysiological parameters derived from nerve conduction studies. Statistical significance of the levels between groups was calculated using unpaired-t test after checking for normality with D'Agostino & Pearson omnibus normality test. RESULTS: We found that the median nerve conduction velocity was prolonged, amplitude was decreased, while the levels of oxidative stress markers like malondialdehyde (MDA), superoxidase dismutase (SOD), and nitric oxide (NO) were increased in CTS patients compared to controls. Inflammatory markers like interleukin-6 (IL-6) and tumor necrosis factor-alpha (TNF-α) were also increased in CTS patients. We found that plasma SOD and TNF-α correlated well with the median motor amplitude. There was no other significant correlation between oxidative stress markers and inflammatory markers with nerve conduction studies or disease severity. Patients with mild disease also showed lesser levels of SOD, NO, IL-6, and TNF-α markers than patients with severe disease. CONCLUSIONS: CTS is probably a disease of sterile inflammation and disbalance of oxidative stress, with higher inflammatory and oxidative stress markers pointing to a more severe disease.

Utility of Preoperative Electrodiagnosis Together with Peripheral Nerve High-Resolution Ultrasound: A Complex Case Report of Neurofibromatosis Type I.

Our case report underscores the importance of electroneuromyography (ENMG) combined with peripheral nerve high-resolution ultrasound (HRUS) in the evaluation of neurofibromatosis type 1 (NF1). A 49-year-old woman affected by NF1 came to our attention because of new-onset left arm weakness and atrophy. Debulking of a cervicothoracic C7-T1 neurofibroma had been performed 8 years earlier. On current admission, magnetic resonance imaging disclosed increased lesion volume that was thought to cause the neurologic deficits by compressing the C8 root. Findings from intraoperative neurophysiologic monitoring during repeat debulking suggested that C8 root integrity had been compromised during the first operation and that the new-onset symptoms probably stemmed from peripheral nervous system damage distal to the cervical roots. Postoperative ENMG showed chronic denervation signs in the muscles innervated by C7-C8-T1 roots, moderate carpal tunnel syndrome (CTS), and ulnar nerve conduction block at the elbow. HRUS confirmed the CTS and revealed multiple neurofibromas involving the distal tract of the radial, ulnar, and median nerves. Surgical debulking was considered unnecessary in this case. ENMG combined with nerve and plexus HRUS evaluation may help identify the cause of neurologic deficits and choose the best surgical option in such complex clinical conditions as NF1.

Structural Origin and Surgical Complications of Peripheral Schwannomas.

BACKGROUND/AIM: In this study, we investigated the locations and surgical complications of schwannomas. PATIENTS AND METHODS: Data of 130 patients with schwannomas were retrospectively reviewed. Pre- and post-operative neurological symptoms, tumor locations, and nerves of origin (sensory, motor, or mixed) were reviewed. RESULTS: Before surgery, 69 patients had Tinel-like signs, 56 patients had pain, 32 patients had numbness, four patients had motor deficits. After surgery, 20 patients had developed a new neurological deficit; 11 patients had motor deficits, ten patients had sensory deficits, and one patient had both motor and sensory deficits. Most schwannomas occurred in mixed nerves, including the median nerve in 17 patients and tibial nerve in 13 patients. CONCLUSION: The most common site of schwannoma was the median nerve. Although the nerve of origin of the schwannoma could be identified in only 26.0% of cases, the data suggest that schwannomas occur in both sensory and motor nerves.

Motor excitability measurements in early stage familial amyloid polyneuropathy: The influence of tafamidis treatment.

OBJECTIVE: To test motor fiber excitability in early affected patients with transthyretin (TTR)-type familial amyloid polyneuropathy (TTR-FAP) before and during tafamidis treatment. METHODS: We examined the left median nerve of 21 healthy-matched controls and 10 early affected TTR-FAP patients using the automated threshold-tracking program, QTRAC. TTR-FAP patients were tested one day before the initiation of tafamidis treatment, 3 and 6 months later. RESULTS: The drug was well-tolerated in all patients; there was no drop-out. No statistical difference was found between healthy controls and TTR-FAP patients at study entry. On treatment, both stimulus intensity for 50% of the maximal motor response and rheobase increased significantly from entry to the last evaluation at 6 months (P<0.05). Strength duration time constant decreased significantly from the 3rd to the 6th month of evaluation (P<0.05). There was also a "fanning-out" effect on the late depolarization phase (TEd 90-100ms) as well as a shortened relative refractory period from study entry to the 6th month of evaluation. CONCLUSIONS: Threshold-tracking of median nerve motor fibers is not a helpful technique for the early diagnosis of TTR-FAP patients. Tafamidis was well-tolerated. We observed possible membrane hyperpolarization during treatment. Threshold tracking can contribute to documenting the action of new drugs to treat neuropathies. Tafamidis may change nerve electrical properties by reducing the burden of amyloid fibrils.

Multifocal, hypoechogenic nerve thickening in Cerebrotendinous Xanthomatosis.

OBJECTIVE: To characterize peripheral nerve morphology in cerebrotendinous xanthomatosis (CTX) patients using high-resolution ultrasound (HRUS) in vivo. We hypothesized that nerve enlargements might be present in CTX as a result of accumulation of abnormal lipids with deposition also in peripheral nerves. METHODS: Four CTX patients were examined using HRUS to assess morphological abnormalities of peripheral nerves as well as cervical nerve roots 5 and 6. RESULTS: HRUS revealed mild to moderate, hypoechogenic thickening of sensorimotor nerves (ulnar nerve in 1/4, tibial nerve in 3/4, median nerve 4/4 patients) as well as mild enlargement of pure sensory nerves (sural nerve in 2/3, superficial FN in 2/4 patients). The vagal nerve was moderately enlarged in one patient, cervical roots showed moderate enlargements of C5 in two patients, one of which also showing thickening of C6 as well as in another patient. UPSS score was slightly to moderately abnormal in all patients. The Homogeneity score was not increased suggesting regional to inhomogeneous nerve enlargement. CONCLUSIONS: HRUS shows multifocal, hypoechogenic nerve thickening of peripheral nerves and nerve roots in CTX. SIGNIFICANCE: HRUS might serve as a valuable, additive and non-invasive bedside tool to assess peripheral nerve morphology in future clinical studies on CTX patients.

Compound muscle action potential scan and MScanFit motor unit number estimation during Wallerian degeneration after nerve transections.

BACKGROUND: Compound muscle action potential (CMAP) scan and MScanFit have been used to understand the consequences of denervation and reinnervation. This study aimed to monitor these parameters during Wallerian degeneration (WD) after acute nerve transections (ANT). METHODS: Beginning after urgent surgery, CMAP scans were recorded at 1-2 day intervals in 12 patients with ANT of the ulnar or median nerves, by stimulating the distal stump (DS). Stimulus intensities (SI), steps, returners, and MScanFit were calculated. Studies were grouped according to the examination time after ANT. Results were compared with those of 27 controls. RESULTS: CMAP amplitudes and MScanFit progressively declined, revealing a positive correlation with one another. SIs were higher in WD groups than controls. Steps appeared or disappeared in follow-up scans. The late WD group had higher returner% than the early WD and control groups. CONCLUSIONS: MScanFit can monitor neuromuscular dysfunction during WD. SIs revealed excitability changes in DS.

Hypertrophied reversed palmaris longus muscle (pseudotumor) of the forearm causing median nerve compression: a case report.

BACKGROUND: The palmaris longus muscle is considered one of the most anatomically variable muscles in the human body. Localized swelling of the forearm due to hypertrophy of the palmaris longus muscle is rare. CASE PRESENTATION: Here, we report a rare case of a 24-year-old Arab man who presented with a painful mass on his forearm with symptoms of median nerve compression. A full radiological assessment was performed, and he was treated conservatively. CONCLUSION: This case confirmed that a hypertrophied palmaris longus muscle can be the cause of swelling on the forearm and should always be considered in the differential diagnosis. With this report, we aimed to increase awareness regarding the unusual variations of palmaris longus muscle and the importance of using radiological investigations to establish a diagnosis.

Screening for Carpal Tunnel Syndrome in Patients With Mucopolysaccharidosis.

Mucopolysaccharidoses (MPSs) are a group of rare lysosomal storage diseases with multisystem manifestations, including carpal tunnel syndrome (CTS). This study comprised a systematic review of literature and hospital guidelines addressing the method and frequency of screening for carpal tunnel syndrome in mucopolysaccharidosis patients and a review of carpal tunnel syndrome in patients seen in the multidisciplinary mucopolysaccharidosis clinic of a pediatric hospital, in order to develop screening recommendations. The literature reported the importance of routine carpal tunnel syndrome screening from early childhood in patients with mucopolysaccharidosis I, II, IV, and VI. Screening methods included physical examination, nerve conduction studies, electromyography, and ultrasonography. Ten of 20 mucopolysaccharidosis patients in our series underwent carpal tunnel syndrome surgery. Given the high incidence of carpal tunnel syndrome at a young age in mucopolysaccharidosis, the authors recommend performing physical examination and obtaining patient and caregiver history for carpal tunnel syndrome every 6 months from the time of mucopolysaccharidosis diagnosis, supplemented by annual nerve conduction studies in cases with poor history or equivocal examination.

Changes in long term peripheral nerve biophysical properties in childhood cancer survivors following neurotoxic chemotherapy.

OBJECTIVE: In the context of increasing numbers of childhood cancer survivors (CCS), this study aimed to enhance understanding of the biophysical basis for long term chemotherapy induced peripheral neuropathy from different chemotherapy agents in CCS. METHODS: Detailed cross-sectional neurophysiological examination, using median nerve axonal excitability studies, alongside clinical assessments, in 103 long term CCS (10.5 ± 0.6 years post-treatment). RESULTS: Cisplatin treated CCS (n = 16) demonstrated multiple sensory axonal excitability changes including increased threshold (P < 0.05), alterations in depolarising and hyperpolarising threshold electrotonus (P < 0.05) and reduction in resting and minimum IV slope (P < 0.01). Vincristine treated CCS (n = 73) were comparable to controls, except for prolonged distal motor latency (P = 0.001). No differences were seen in the non-neurotoxic chemotherapy group (n = 14). Abnormalities were more evident in the cisplatin subgroup with greater clinical neuropathy manifestations. CONCLUSION: Persistent long term changes in axonal biophysical properties vary with different chemotherapy agents, most evident after cisplatin exposure. Longitudinal studies of nerve function during chemotherapy treatment are required to further evaluate these differences and their mechanistic basis. SIGNIFICANCE: This study provides a unique biophysical perspective for persistent cisplatin related neurotoxicity in children, previously under recognised.

Electrophysiological Evaluation and Clinical Implication of Martin-Gruber Anastomosis in Healthy Subjects.

Background: The aim of this study is to determine the prevalence of Martin-Gruber Anastomosis (MGA) in healthy Egyptian subjects, and to discuss the available literature regarding MGA subtypes and their clinical implications. Methods: An electrophysiological study was conducted in both forearms of 140 healthy subjects. This included the ulnar and median nerves. Compound muscle action potentials were recorded from abductor pollicis brevis, abductor digiti minimi, and first dorsal interosseous muscles. Other measurements included the compound motor action potential amplitude and its innervation ratio. Results: MGA was found in 56 of the 280 forearms. This included 20 men and 36 women. Type II MGA subtype was the most frequent in both genders. The MGA was bilateral in 6 subjects and more frequent on the right side. The highest mean amplitude and innervation ratio were recorded at first dorsal interosseus muscle. Conclusions: The prevalence of MGA in the studied sample of the Egyptian population is 20%. It is important for Hand, Orthopaedic and Neurosurgeons to be aware of this anatomic variation in order to explain paradoxical motor and sensory loss in patients.

Neuromuscular ultrasound for taxane peripheral neuropathy in breast cancer.

BACKGROUND: Our study aim was to evaluate neuromuscular ultrasound (NMUS) for the assessment of taxane chemotherapy-induced peripheral neuropathy (CIPN), the dose-limiting toxicity of this agent. METHODS: This cross-sectional study of breast cancer patients with taxane CIPN measured nerve cross-sectional area (CSA) by NMUS and compared with healthy historical controls. Correlations were determined between CSA and symptom scale, nerve conduction studies, and intraepidermal nerve fiber density (IENFD). RESULTS: A total of 20 participants reported moderate CIPN symptoms at a median of 3.8 months following the last taxane dose. Sural nerve CSA was 1.2 mm2 smaller than healthy controls (P ≤ .01). Older age and time since taxane were associated with smaller sural nerve CSA. For each 1 mm2 decrease in sural nerve CSA, distal IENFD decreased by 2.1 nerve/mm (R2 0.30; P = .04). CONCLUSIONS: These data support a sensory predominant taxane neuropathy or neuronopathy and warrant future research on longitudinal NMUS assessment of CIPN.

Postoperative diagnostic potentials of median nerve strain and applied pressure measurement after carpal tunnel release.

BAKGROUND: The objective of this study is to investigate the prognostic values of median nerve strain and applied pressure measurement for the assessment of clinical recovery after carpal tunnel release. METHODS: Forty-five wrists, from 45 idiopathic carpal tunnel syndrome patients who treated with open carpal tunnel release, were evaluated by ultrasound. Median nerve strain, pressure applied to the skin, and ratio of pressure-strain were measured at the proximal part of the carpal tunnel. In addition, distal latencies in the motor and sensory nerve conductions studies and cross-sectional area of median nerve were measured. The parameters were compared before and after the open carpal tunnel release. According to patient recovery, the receiver operating characteristic curves were generated to evaluate the prognostic values of the parameters. The areas under the receiver operating characteristic curves were compared among parameters. RESULTS: There was a significant increase in the median nerve strain, and significant decreases in the pressure applied to the skin and ratio of pressure-strain after carpal tunnel release (P < 0.01). There were significant decreases in the distal latencies and the cross-sectional area after carpal tunnel release (P < 0.01). The areas under the curves were 0.689, 0.773, 0.811, 0.668, 0.637, and 0.562 for the pressure, strain, pressure-strain ratio, motor latency, sensory latency, and area, respectively. CONCLUSIONS: The results suggest that elasticity of the median nerve and pressure around the nerve recover quickly after carpal tunnel release. Pressure-strain ratio was the most reliable parameter to reflect clinical recovery. The measurement of strain and applied pressure can be useful indicators to evaluate effectiveness of the carpal tunnel release. TRIAL REGISTRATION: Registered as NCT04027998 at ClinicalTrials.gov. Retrospectively registered on July 22, 2019.

Visualization of electrophysiological activity at the carpal tunnel area using magnetoneurography.

OBJECTIVE: To establish a noninvasive method to measure the neuromagnetic fields of the median nerve at the carpal tunnel after electrical digital nerve stimulation and evaluate peripheral nerve function. METHODS: Using a vector-type biomagnetometer system with a superconducting quantum interference device, neuromagnetic fields at the carpal tunnel were recorded after electrical stimulation of the index or middle digital nerve in five healthy volunteers. A novel technique for removing stimulus-induced artifacts was applied, and current distributions were calculated using a spatial filter algorithm and superimposed on X-ray. RESULTS: A neuromagnetic field propagating from the palm to the carpal tunnel was observed in all participants. Current distributions estimated from the magnetic fields had five components: leading and trailing components parallel to the conduction pathway, outward current preceding the leading component, inward currents between the leading and trailing components, and outward current following the trailing component. The conduction velocity and peak latency of the inward current agreed well with those of sensory nerve action potentials. CONCLUSION: Removing stimulus-induced artifacts enabled magnetoneurography to noninvasively visualize with high spatial resolution the electrophysiological neural activity from the palm to the carpal tunnel. SIGNIFICANCE: This is the first report of using magnetoneurography to visualize electrophysiological nerve activity at the palm and carpal tunnel.

A Model to Study Myelinated Fiber Degeneration and Regeneration in Human Skin.

OBJECTIVE: To describe morphological changes associated with degeneration and regeneration of large fibers in the skin using a model of chronic compression of the median nerve. METHODS: We studied cutaneous innervation in 30 patients with chronic compression of the median nerve at the wrist. Before surgery, we assessed the symptom severity and performed neurography, quantitative sensory testing, and analysis of nerve morphology and morphometry in skin biopsies from the third digit fingertip. Fifteen patients repeated all tests 12 months after the surgery. Thirty age- and sex-matched healthy subjects were included in the study. RESULTS: Clinical and neurophysiological basal assessment showed a moderate involvement of the median nerve. Quantitative sensory testing showed abnormal findings. The density of intraepidermal nerve fibers and intrapapillary myelinated endings was reduced. Myelinated fibers showed caliber reduction and nodal elongation. Meissner corpuscles had normal density but were located deeper in the dermis and their capsule appeared partially empty. During follow-up, patients exhibited a positive clinical and neurophysiological outcome. Quantitative sensory testing improved. Intraepidermal nerve fibers and intrapapillary myelinated endings remained unchanged, but the caliber of intrapapillary myelinated endings was increased. The neural component of the Meissner corpuscle filled the capsule of the mechanoreceptors that remained deeper in the dermis. The position of vasoactive intestinal peptide-immunoreactive fibers was more superficial compared to the basal assessment and controls. INTERPRETATION: We recognized and quantified the pathological changes associated with nerve degeneration and regeneration in skin and proposed new parameters that may increase the diagnostic yield of skin biopsy in clinical practice. Ann Neurol 2020;87:456-465.

Prospective Evaluation of Median Nerve Dysfunctions in Patients with a Distal Radius Fracture Treated with Volar Locking Plating.

Background: The aim of this study was to identify the risk factors for median nerve dysfunctions after volar locking plate (VLP) fixation for distal radius fracture (DRF). Methods: We prospectively assessed the incidence of median nerve symptoms (MNS) such as numbness, pain, paresthesia, or hypesthesia in the area innervated by the median nerve and evaluated post-operative nerve conduction (NC) in 91 hands of 121 patients after VLP fixation for DRF. Multivariate logistic regression analysis was conducted to identify factors independently associated with MNS and abnormal NC in the injured wrist. Results: There were 18 cases (20%) of MNS on the injured side, 9 hands (10%) of both MNS and abnormal NC, 11 hands (12%) with only abnormal NC, and 9 hands with only MNS. Sensitivity, specificity, and diagnostic accuracy of abnormal NC for diagnosing MNS were 50%, 86%, and 78%, respectively. Four cases did not respond to conservative treatment and received carpal tunnel release concomitantly with plate removal. Logistic regression examination revealed that volar placement of the plate and short stature were significant independent predictors of MNS, while patient age was the sole independent predictor of abnormal NC. Conclusions: Our study demonstrated that plate prominence, short stature, and age were significant independent risk factors for median nerve dysfunctions after VLP fixation for DRF.

Consideration of repetitive nerve stimulation of the median nerve in patients being evaluated for myasthenia gravis.

Repetitive nerve stimulation (RNS) of the median nerve is rarely studied in myasthenia gravis (MG). We performed a retrospective analysis of RNS studies performed on 448 patients at our center between 2010 and 2016. Among 95 patients with MG, an abnormal decrement of the compound muscle action potential amplitude was seen in 40.7% of median, 38.4% of spinal accessory, and 57.1% of facial nerve RNS studies. Median nerve RNS was abnormal in 10 patients with normal spinal accessory and 5 patients with normal facial nerve RNS. MG patients with abnormal median nerve RNS were more likely to be seropositive and have limb weakness. No differences were observed in the incidence of abnormal median nerve RNS in patients with and without a median neuropathy at the wrist. Median nerve RNS is easy to perform, reproducible, and should be considered in the electrodiagnostic evaluation of MG.