OBJECTIVE: To analyze and compare the characteristics and causes of F wave changes in patients with Charcot-Marie-Tooth1A (CMT1A) and chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Thirty patients with CMT1A and 30 patients with CIDP were enrolled in Peking University Third Hospital from January 2012 to December 2018. Their clinical data, electrophysiological data(nerve conduction velocity, F wave and H reflex) and neurological function scores were recorded. Some patients underwent magnetic resonance imaging of brachial plexus and lumbar plexus, and the results were analyzed and compared. RESULTS: The average motor conduction velocity (MCV) of median nerve was (21.10±10.60) m/s in CMT1A and (31.52±12.46) m/s in CIDP. There was a significant difference between the two groups (t=-6.75, P < 0.001). About 43.3% (13/30) of the patients with CMT1A did not elicit F wave in ulnar nerve, which was significantly higher than that of the patients with CIDP (4/30, 13.3%), χ²=6.65, P=0.010. Among the patients who could elicit F wave, the latency of F wave in CMT1A group was (52.40±17.56) ms and that in CIDP group was (42.20±12.73) ms. There was a significant difference between the two groups (t=2.96, P=0.006). The occurrence rate of F wave in CMT1A group was 34.6%±39%, and that in CIDP group was 70.7%±15.2%. There was a significant difference between the two groups (t=-5.13, P < 0.001). The MCV of median nerve in a patient with anti neurofascin 155 (NF155) was 23.22 m/s, the latency of F wave was 62.9-70.7 ms, and the occurrence rate was 85%-95%. The proportion of brachial plexus and lumbar plexus thickening in CMT1A was 83.3% (5/6) and 85.7% (6/7), respectively. The proportion of brachial plexus and lumbar plexus thickening in the CIDP patients was only 25.0% (1/4, 2/8). The nerve roots of brachial plexus and lumbar plexus were significantly thickened in a patient with anti NF155 antibody. CONCLUSION The prolonged latency of F wave in patients with CMT1A reflects the homogenous changes in both proximal and distal peripheral nerves, which can be used as a method to differentiate the CIDP patients characterized by focal demyelinating pathology. Moreover, attention should be paid to differentiate it from the peripheral neuropathy caused by anti NF155 CIDP. Although F wave is often used as an indicator of proximal nerve injury, motor neuron excitability, anterior horn cells, and motor nerve myelin sheath lesions can affect its latency and occurrence rate. F wave abnormalities need to be comprehensively analyzed in combination with the etiology, other electrophysiological results, and MRI imaging.
Humans ; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/pathology* ; Median Nerve/pathology* ; Ulnar Nerve/pathology* ; Brachial Plexus/pathology* ; Magnetic Resonance Imaging/methods*

Melorheostosis is a rare, progressive bone disease accompanied by hyperostosis and soft tissue fibrosis. While affected adults present with contracture and pain, children present with limb length discrepancy and deformity. We report the case of a 20-year-old woman with melorheostosis since childhood who presented with right hand deformity and numbness. Radiographs showed not only a combination of dense sclerosis and opacities, but also the classic 'flowing candle wax' appearance. Radiography can be used to identify melorheostosis, thus preventing unnecessary bone biopsies. Carpal tunnel release revealed the presence of a thickened flexor retinaculum and a degenerated median nerve distal to the retinaculum, but did not show hyperostosis. This case highlights the role of nerve decompression in melorheostosis and the importance of early identification of the disease to prevent unnecessary bone biopsies.
Carpal Tunnel Syndrome ; complications ; diagnostic imaging ; surgery ; Diagnosis, Differential ; Female ; Fibrosis ; pathology ; Hand ; pathology ; Hand Deformities ; diagnostic imaging ; surgery ; Humans ; Hypesthesia ; Median Nerve ; surgery ; Melorheostosis ; complications ; diagnostic imaging ; surgery ; Radiography ; Sclerosis ; diagnostic imaging ; Young Adult

OBJECTIVETo study the clinicopathologic features of lipomatosis of nerve (NLS).

METHODSThe clinical, radiologic and pathologic features were analyzed in 15 cases of NLS.

RESULTSThere were a total of 10 males and 5 females. The age of patients ranged from 4 to 42 years (mean age = 22.4 years). Eleven cases were located in the upper limbs and 4 cases in the lower limbs. The median nerve was the most common involved nerve. The patients typically presented before 30 years of age (often at birth or in early childhood) with a soft and slowly enlarging mass in the limb, with or without accompanying motor and sensory deficits. Some cases also had macrodactyly and carpal tunnel syndrome. MRI showed the presence of fatty tissue between nerve fascicles, resembling coaxial cable in axial plane and assuming a spaghetti-like appearance in coronal plane. On gross examination, the affected nerve was markedly increased in length and diameter. It consisted of a diffusely enlarged greyish-yellow lobulated fusiform beaded mass within the epineural sheath. Histologically, the epineurium was infiltrated by fibrofatty tissue which separated, surrounded and compressed the usually normal-appearing nerve fascicles, resulting in perineural septation of nerve fascicles and microfascicle formation. The infiltration sometimes resulted in concentric arrangement of perineural cells and pseudo-onion bulb-like hypertrophic changes. The perineurial cells might proliferate, with thickening of collagen fibers, degeneration and atrophic changes of nerve bundles. Immunohistochemical study showed that the nerve fibers expressed S-100 protein, neurofilament and CD56 (weak). The endothelial cells and dendritic fibers were highlighted by CD34. The intravascular smooth muscle cells were positive for muscle-specific actin.

CONCLUSIONSNLS is a rare benign soft tissue tumor of peripheral nerve. The MRI findings are characteristic. A definitive diagnosis can be made with histologic examination of tissue biopsy.

Adolescent ; Adult ; Antigens, CD34 ; metabolism ; CD56 Antigen ; metabolism ; Carpal Tunnel Syndrome ; complications ; pathology ; Child ; Child, Preschool ; Diagnosis, Differential ; Extremities ; innervation ; Female ; Hand Deformities, Congenital ; complications ; pathology ; Humans ; Lipoma ; pathology ; Lipomatosis ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Magnetic Resonance Imaging ; Male ; Median Nerve ; metabolism ; pathology ; Nerve Sheath Neoplasms ; pathology ; Neurofibroma ; pathology ; Neurofilament Proteins ; metabolism ; Neuroma ; pathology ; Peripheral Nervous System Diseases ; complications ; diagnosis ; metabolism ; pathology ; surgery ; Peripheral Nervous System Neoplasms ; pathology ; Retrospective Studies ; S100 Proteins ; metabolism ; Vimentin ; metabolism ; Young Adult

Antigens, CD34 ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangioma ; metabolism ; pathology ; surgery ; Hemangiosarcoma ; pathology ; Humans ; Median Nerve ; pathology ; Median Neuropathy ; metabolism ; pathology ; surgery ; Melanoma ; pathology ; Middle Aged ; Peripheral Nervous System Neoplasms ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism

BACKGROUND: The aim of this study was to evaluate the contribution of the proximal nerve stump, in end-to-side nerve repair, to functional recovery, by modifying the classic end-to-side neurorrhaphy and suturing the proximal nerve stump to a donor nerve in a rat model of a severed median nerve. METHODS: Three experimental groups were studied: a modified end-to-side neurorrhaphy with suturing of the proximal nerve stump (double end-to-side neurorrhaphy, Group I), a classic end-to-side neurorrhaphy (Group II) and a control group without neurorrhaphy (Group III). Twenty weeks after surgery, grasping testing, muscle contractility testing, and histological studies were performed. RESULTS: The grasping strength, muscle contraction force and nerve fiber count were significantly higher in group I than in group II, and there was no evidence of nerve recovery in group III. CONCLUSIONS: The contribution from the proximal nerve stump in double end-to-side nerve repair might improve axonal sprouting from the donor nerve and help achieve a better functional recovery in an end-to-side coaptation model.
Anastomosis, Surgical/methods ; Animals ; Axons/pathology ; Forelimb ; Hand Strength ; Male ; Median Nerve/pathology/*surgery ; Muscle Contraction ; Muscle, Skeletal/physiopathology ; Nerve Regeneration ; Nerve Transfer/*methods ; Rats ; Rats, Sprague-Dawley ; Recovery of Function ; Ulnar Nerve/pathology/*surgery

OBJECTIVETo study the method and effect of endoscopic two-portal one-way releasing procedure for cut of transverse carpal ligament and decompression of median nerve.

METHODSEleven female patients (13 sides) with primary carpal tunnel synrome underwent endoscopic two-portal one-way releasing procedure, there were 3 left hands, 6 right, and 2 both. All the subjects had hypesthesia in the radial three and half finger's tip with a positive, Tinel sign of median nerve at wrist; 11 cases had thenar myatrophy in which 4 had opposition dysfunction. Under local anaesthesia, the proximal incision was located at the point of the proximal carpal transverse striation level between palmaris longus and flexor carpi radialis. The outlet was chosed the junction of the parallel line of the ulnar side of thumb and proximal extending line of middle ring fingers' long axis while the thumb was in abduction position. The length of each incision was only one centimeter. The hook knife was inserted to the proximate of the transverse carpal ligament, then, the transverse carpal ligament was completely released form the proximal to the distal end by hook knife under the endoscope monitor.

RESULTSThe results showed that both pinch and grip function was satisfied and no complications occurred at 4 to 20 months followed-up. S3+ M3 or more has been reached in 3 months after operation.

CONCLUSIONThe endoscopic two-portal one-way releasing procedure is an easy and effective method for the treatment of carpal tunnel syndrome.

Adult ; Aged ; Carpal Tunnel Syndrome ; pathology ; physiopathology ; surgery ; Decompression, Surgical ; Endoscopy ; methods ; Female ; Follow-Up Studies ; Humans ; Ligaments ; surgery ; Median Nerve ; surgery ; Middle Aged ; Recovery of Function

OBJECTIVE: This study aimed to clarify the morphology of the Martin-Gruber anastomosis (MGA) in Chinese. METHODS: One hundred and five Chinese upper limbs (36 males and 20 femalese) were dissected to find the connections between medial nerve and ulnar nerve. The MGA was classified as previously described by Lee. RESULTS: MGA was found in 24 cases (22.9%), in 11 of the 36 male and 5 of the 20 female. There was no obvious difference in the frequency of MGA in both upper limbs. Most MGA ulnar position was located at the medial and distal segment of the forearm. CONCLUSION MGA anatomy could play important role in forensic diagnosis of ulnar nerve injury in Chinese population.
Cadaver ; China/epidemiology* ; Expert Testimony/legislation & jurisprudence* ; Female ; Humans ; Male ; Median Nerve/pathology* ; Muscle, Skeletal/innervation* ; Nervous System Malformations/physiopathology* ; Ulnar Nerve/pathology* ; Upper Extremity/innervation*

BACKGROUND: The femoral artery is the most common site of cannulation for cardiopulmonary bypass in surgery for type A aortic dissection. Recently, many surgeons prefer the axillary artery to the femoral artery as the arterial cannulation site for several benefits. We evaluated the safety and usefulness of axillary artery cannulation in surgery for acute type A aortic dissection. MATERIAL AND METHOD: Between Oct. 1995 and Sep. 2001, 71 patients underwent operations for acute type A aortic dissection. The arterial cannula was inserted into the axillary artery in 31 patients (AXILLARY group, mean age=56), and into the femoral artery in 40 patients (FEMORAL group, mean age=57). We retrospectively compared the incidence of mortality, morbidities, and hospital course. RESULT: The mean duration of cardiopulmonary bypass and circulatory arrest were significantly shorter in the AXILLARY group (207 min and 39min, respectively) than in the FEMORAL group (263 min and 49 min, respectively; p<0.05). Postoperative hospital stay was significantly shorter in the AXILLARY group than in the FEMORAL group (mean 15 days vs. 35 days, p<0.05). Although there was no difference in the incidence of new-onset permanent neurological dysfunction (3.2% in the AXILLARY group, 2.5% in the FEMORAL group), the incidence of transient neurological dysfunction was significantly lower in the AXILLARY group (12.9% vs. 25%, p<0.05). In the FEMORAL group, two patients needed urgent conversion to cannulation site due to arch vessel malperfusion. In the AXILLARY group, there was only one patient who had a complication related to the cannulation, i.e., median nerve injury. CONCLUSION: Axillary artery cannulation was safe and helpful in decreasing the cerebral ischemic time and incidence of transient neuroligcal dysfunction in surgery for acute type A aortic dissection. It enabled us to approach the patients with aortic arch pathology more aggressively.
Aneurysm, Dissecting ; Aorta, Thoracic ; Axillary Artery* ; Cardiopulmonary Bypass ; Catheterization* ; Catheters ; Femoral Artery ; Humans ; Incidence ; Length of Stay ; Median Nerve ; Mortality ; Pathology ; Retrospective Studies

Leprosy, one of the oldest diseases known to humans, still affects almost 10-15 million people throughout the world. Neuropathy affects 15-20% of infected individuals. Therefore leprous neuropathy is still one of the most common neuropathy in the world. It is due to infection with Mycobacterium leprae and occurs primarily in Asia and Africa. The cardinal symptom of leprosy is sensory loss caused by superficial neuropathy. Anesthetic depigmented skin lesions are an important finding and should be sought. Nerve involvement is a hallmark of leprosy. Nerve involvement is rare in other diseases, so the finding of skin lesions with enlarged nerves should raise the possibility of leprosy. Nerve involvement tends to occur with skin lesions, and the pattern of nerve involvement parrellels the skin disease. In the tuberculoid form, mononeuropathy multiplex is typical pattern, whereas asymmetrical or symmetrical polyneuropathy is most common in the lepromatous form. Motor involvement occurs in a predictable sequence as a result of nerve trunk damage to those nerves that course close to the skin surface and hence are locally cool. Nerve involved include the ulnar nerve at the elbow, the deep peroneal branch at the ankle, superficial branches of the facial nerve, and the median nerve at the wrist, and especially the greater auricular nerve. Nerve involvement without skin lesions, called pure neural leprosy, can occur. Other characteristic findings are thickened nerve, trophic ulcers, mutilated digits, and Charcot joint. In clinically affected nerves, the motor nerve conduction velocities are minimally slow. The terminal latency is often prolonged and the compound muscle action potentials are temporally dispersed and decreased in amplitude. Sensory and mixed compound nerve action potentials are often difficult to obtain or else a reduced amplitude. The facial nerve is commonly involved in leprosy. Improvement in motor nerve conduction was reported in leprosy patients under sulfone treatment. Nerve biopsy reveals subperineurial edema and various amounts of loss of myelinated and unmyelinated fibers. Teased fiber studies reveal paranodal demyelination affecting successive internodes. Therefore segmental demyelination is the main pathology in leprous neuropathy.
Action Potentials ; Africa ; Ankle ; Arthropathy, Neurogenic ; Asia ; Biopsy ; Demyelinating Diseases ; Edema ; Elbow ; Facial Nerve ; Humans ; Leprosy ; Leprosy, Tuberculoid ; Median Nerve ; Mononeuropathies ; Mycobacterium leprae ; Myelin Sheath ; Neural Conduction ; Pathology ; Polyneuropathies ; Skin ; Skin Diseases ; Ulcer ; Ulnar Nerve ; Wrist

BACKGROUND: Topographic mappings of somatosensory evoked potentials (SEP) on subdural electrodes help identify the motor cortex quickly during chronic subdural recordings or during the operation. We tried to assess the ease and reliability of the routine use of SEP for identification of the sensorimotor cortex depending on pathology and location of the lesion. METHODS: We reviewed 75 SEP studies of 63 patients who needed functional mappings of the sensorimotor area. The phase reversal (PR) of SEP around the 20 msec latency in response to contralateral median nerve stimulations by subdural electrodes was used to identify the position of the central sulcus. The patients included 20 with nonlesional epilepsy, 30 with tumor, 12 with arteriovenous malformation (AVM), and 1 with cavernous angioma. RESULTS: SEP-PRs were successfully recorded in 67 SEP among 75 studies (89.3%). SEP-PRs were recorded in 37 of 43 patients with lesions (86.0%), and in all patients without lesions (100.0%). In regards to pathology, the absence of SEP-PR was noted in 3 out of 12 patients with AVM (25.0%), 3 out of 30 patients with tumor (10.0%), and 0 out of 1 patient with cav-ernous angioma (0.0%). The SEP-PRs were obtained the least frequently for the location of lesions when the lesions involved both the frontal and parietal areas. CONCLUSIONS: Intraoperatively, the SEP-PR can be easily obtained and the median nerve SEP is an useful test for confirming the identification of the central sulcus. SEP-PR can be detected more frequently in patients without lesions rather than in those with lesions, especially patients with AVM or whose lesions are over the frontoparietal areas.
Arteriovenous Malformations ; Brain Mapping ; Electrodes* ; Epilepsy ; Evoked Potentials ; Evoked Potentials, Somatosensory* ; Hemangioma ; Hemangioma, Cavernous ; Humans ; Median Nerve ; Motor Cortex ; Neurosurgery ; Pathology ; Rabeprazole