Pronator syndrome and anterior interosseous nerve palsy due to neurolymphomatosis: a case report.

Pronator syndrome is a median nerve entrapment neuropathy that can be difficult to diagnose due to its variable presentation and objective findings. Neurolymphomatosis is an uncommon disease in which malignant lymphocytes infiltrate central or peripheral nerve endoneurium and is often missed for prolonged periods prior to diagnosis. We present a rare case of pronator syndrome and anterior interosseous nerve palsy due to neurolymphomatosis that was occult on initial MRI in spite of the presence of a median nerve mass discovered intra-operatively during neurolysis. This case demonstrates the value of ultrasound for the examination of peripheral nerve pathology and illustrates its utility as an adjunct to MRI, in part due to the ability to screen a large region.

Low-grade fibromyxoid sarcoma arising within the median nerve.

We report a case of low-grade fibromyxoid sarcoma arising within the median nerve. A 31-year-old woman presented with symptoms of carpal tunnel syndrome and an enlarging mass in her right palm over 1 year. MRI demonstrated a mass associated with the right median nerve with solid and cystic components. At surgery, the mass was located within the epineurium, could be bluntly dissected from the nerve fascicles, and was suspected to be a schwannoma. A 3.4 cm, tan-pink, glistening, smooth, homogenous mass was submitted to pathology. Microscopically, the tumor was a solid and cystic circumscribed nodule with a dense fibrous pseudocapsule. The tumor cells were uniformly bland and spindle-shaped, with small, hyperchromatic oval nuclei and were embedded in an alternating fibrous and myxoid stroma with a prominent curvilinear vasculature and perivascular sclerosis. The differential diagnosis for this lesion included myxoid neurofibroma, schwannoma, soft tissue perineurioma, low-grade malignant peripheral nerve sheath tumor and low-grade fibromyxoid sarcoma. The tumor cells expressed MUC4, GLUT-1, and vimentin and were negative for S-100 protein, epithelial membrane antigen, smooth muscle actin, desmin, claudin-1, neurofilament and SOX10. Fluorescence in situ hybridization, with a break-apart probe strategy, demonstrated FUS rearrangement, consistent in this morphological context with the low-grade fibromyxoid sarcoma-associated FUS-CREB3L2 or FUS-CREB3L1 fusions. Low-grade fibromyxoid sarcoma is exceptionally rare in the peripheral nerve, with only a single previously reported case. Nonetheless, as our case illustrates, this entity must be included in the differential diagnosis of unusual intraneural mesenchymal tumors. As in all other locations, intraneural low-grade fibromyxoid sarcomas should be excised with negative margins. Patients with this disease require long-term clinical follow-up, given this tumor's propensity for very late distant metastases to the lungs and other sites.

Cortical plasticity in patients with median nerve lesions studied with MEG.

We have previously shown age- and time-dependent effects on brain activity in the primary somatosensory cortex (SI), in a functional magnetic resonance imaging (fMRI) study of patients with median nerve injury. Whereas fMRI measures the hemodynamic changes in response to increased neural activity, magnetoencephalography (MEG) offers a more concise way of examining the evoked response, with superior temporal resolution. We therefore wanted to combine these imaging techniques to gain additional knowledge of the plasticity processes in response to median nerve injury. Nine patients with median nerve trauma at the wrist were examined with MEG. The N1 and P1 responses at stimulation of the injured median nerve at the wrist were lower in amplitude compared to the healthy side (p < .04). Ulnar nerve stimulation of the injured hand resulted in larger N1 amplitude (p < .04). The amplitude and latency of the response did not correlate with the sensory discrimination ability. There was no correlation between N1 amplitude and size of cortical activation in fMRI. There was no significant difference in N1 latency between the injured and healthy median nerve. N1 latency correlated positively with age in both the median and ulnar nerve, and in both the injured and the healthy hand (p < .02 or p < .001). It is concluded that conduction failure in the injured segment of the median nerve decreases the amplitude of the MEG response. Disinhibition of neighboring cortical areas may explain the increased MEG response amplitude to ulnar nerve stimulation. This can be interpreted as a sign of brain plasticity.

Platelet-rich plasma limits the nerve injury caused by 10% dextrose in the rabbit median nerve.

INTRODUCTION: We evaluated the effect of platelet-rich plasma (PRP) injection in a rabbit model of dextrose-induced median nerve injury. METHODS: New Zealand white rabbits (n = 15) were divided randomly into 3 groups. Three different regimens (group 1: 0.1 ml saline; group 2: 10% dextrose with PRP; group 3: 10% dextrose with saline) were injected within the carpal tunnel. Electrophysiological and histological findings were evaluated 12 weeks after the injection. RESULTS: The mean median motor latency in group 3 was significantly longer than that in groups 1 and 2. The cross-sectional area of the median nerve and subsynovial connective tissue thickness in group 3 were significantly larger than those in groups 1 and 2. CONCLUSIONS: PRP injection may be effective in controlling median nerve injury, as demonstrated by improvement in electrophysiological and histological findings 12 weeks after dextrose injection.

Isolated median neuropathy as the first symptom of leprosy.

INTRODUCTION: Focal peripheral neuropathy of the median nerve is mainly caused by a traumatic event or pressure, but it may also be produced by systemic illnesses. Among the latter, leprosy is a rare cause. METHODS: Six cases of isolated median neuropathy as the first sign of leprosy were selected from patients with an exclusively neurological complaint as the initial symptom. The patients, evaluated at the National Leprosy Reference Center in Rio de Janeiro, Brazil, followed routine and specialized procedures. RESULTS: Three of the patients had pure neural leprosy, and 3 had skin lesions. Clinical median nerve function impairment was confirmed by neurophysiological testing and histopathology. Both mononeuritis and mononeuritis multiplex were observed. CONCLUSIONS: This case series demonstrates an additional form of presentation of leprosy, which, if not diagnosed and treated in time, may lead to permanent disability.

Relating applied strain to the type and severity of structural damage in the rat median nerve using second harmonic generation microscopy.

INTRODUCTION: Stretch injuries in peripheral nerves can cause pain, paralysis, and loss of sensation. Although optimal treatment depends on the degree of injury, it is difficult to determine the severity of induced nerve damage. METHODS: The load-deformation curves of rat median nerves were generated from monotonic load-to-failure experiments to determine low, medium, and high strain levels. Additional excised median nerves were then elongated to induce damage at low (4%), medium (10% and 12%), and high (14% and 20%) tensile strains and the resulting structural damage was evaluated using second harmonic generation (SHG) imaging and light microscopy. RESULTS: No substantial structural changes occurred at 4% strain, but higher strain values resulted in disruption of the normal collagen architecture. CONCLUSIONS: The results demonstrate a spectrum of structural damage that can be monitored using SHG, a non-destructive imaging modality, and that the pattern of damage may correspond to functional deficit.

Diagnostic biopsy of the pronator teres and a motor branch of the median nerve: indications and technique.

PURPOSE: Biopsy of muscle tissue and motor nerve is helpful in the neurological evaluation of patients who present with upper limb and/or diffuse motor weakness. The procedure is indicated to supplement clinical, serological, and imaging diagnostic work-up of myopathic and neuropathic disorders. We describe a surgical technique and clinical series of biopsy of the pronator teres muscle and a motor branch of the median nerve. METHODS: We performed a retrospective review of 20 patients who underwent biopsy of the pronator teres and a motor branch of the median nerve as part of a clinical, serological, and radiographic evaluation for weakness of the upper extremity. All of the biopsies were performed by a single surgeon. The surgical technique is described. Follow-up visits with both the surgeon and the neurologist were reviewed to evaluate preoperative and postoperative neurological function to identify any changes in nerve or muscle function and any postoperative complications. RESULTS: Biopsied tissue was sufficient for pathological diagnosis in all 20 patients. Diagnoses included multifocal motor neuropathy in 14 patients, amyotrophic lateral sclerosis in 3 patients (2 sporadic; 1 familial), inclusion body myositis (1 patient), inflammatory myopathy (1 patient), and chronic inflammatory demyelinating polyneuropathy (1 patient). At a mean follow-up of 11 weeks (range, 5-31 wk), there were 6 minor surgical complications, all of which were superficial hematomas that resolved with use of a compressive wrap. CONCLUSIONS: Biopsy of the pronator teres and a motor branch of the median nerve was safe and effective. The technique is particularly useful when considering the diagnosis of multifocal motor neuropathy affecting the upper extremity.

Neurothekeoma of the median nerve: case report.

We report the case of a large intraneural neurothekeoma of the median nerve at the wrist. Neurothekeomas are rare; they are small, superficial, and typically asymptomatic benign tumors of undetermined cellular origin. Complete excision is usually curative. This case is interesting owing to the tumor's large size and location within the median nerve, which made it highly symptomatic, mimicking carpal tunnel syndrome.

Anterior interosseous nerve compression syndrome: is there a role for endoscopy?

BACKGROUND: Anterior interosseous nerve syndrome is characterized by paralysis of the flexor digitorum profundus, the flexor pollicis longus and the pronator quadratus muscles without sensory loss. Extended exploration of the anterior interosseous nerve is the surgical treatment of choice. The present study evaluates the feasibility of an endoscopic approach for nerve decompression. METHODS: Preparation of the anterior interosseous nerve was performed in ten human cadaver arms. Subsequently, one female patient suffering from anterior interosseous nerve syndrome was endoscopically operated on. FINDINGS: A skin incision of 3-4 cm in the proximal direction was made at the forearm, and the median nerve was visualized between the pronator teres muscle and the flexor digitorum superficialis. Subsequently, the anterior interosseus nerve branch was identified, followed distally and decompressed under endoscopic view. The procedure could be accomplished in all cases under endoscopic view. Due to the very steep surgical angle, a branch of the anterior interosseus nerve was injured in one cadaver case. In all other cases, no adverse effects were observed. In the clinical case, the anterior interosseus nerve was endoscopically identified and decompressed, but a skin incision of 5 cm was required. CONCLUSIONS: The results demonstrate that an endoscopic decompression of the anterior interosseus nerve is possible. Several difficulties occurred: Due to the depth of the surgical approach, especially in case of bulky muscles and very small skin incisions, the view is limited, harboring a higher risk of nerve injury. With more experience and specially designed endoscopes, application of this technique in anterior interosseus nerve compression syndrome might become more feasible.

Cadaveric study of median nerve entrapment in the arm: report of two anatomical cases.

The authors report two anatomic cases of median nerve entrapment, which can be one of the causes of carpal tunnel syndrome. Both cases were soft tissue thickening on the distal arm. The first case was the thickening of brachial fascia that resembles the Struther's ligament. The second case was the thickening of the bicipital aponeurosis combined with the supernumerary biceps brachii. Both cases demonstrated the possible cause of median nerve entrapment at the arm, which mimicked the carpal tunnel syndrome that normally occurs at the wrist. The study reports other possibly sites of causes of median nerve entrapment that clinicians should be aware of the median nerve in the arm proximal to the wrist where the carpal tunnel syndrome normally occurs. These are other points of medina nerve entrapment that clinicians should aware.

Rho-associated kinase II (ROCKII) limits axonal growth after trauma within the adult mouse spinal cord.

Rho GTPases are thought to mediate the action of several axonal growth inhibitors in the adult brain and spinal cord. RhoA has been targeted pharmacologically in both humans and animals to promote neurite outgrowth and functional recovery following CNS trauma. However, rat spinal cord injury studies suggest a complicated and partial benefit of inhibiting Rho or its downstream effector, Rho-associated kinase (ROCKII). This limited benefit may reflect inhibition of other kinases, poor access, or a minimal role of ROCKII in vivo. Therefore, we studied ROCKII mutant mice to probe this pathway genetically. ROCKII(-/-) dorsal root ganglion neurons are less sensitive to inhibition by Nogo protein or by chondroitin sulfate proteoglycan in vitro. We examined adult ROCKII(-/-) mice in two injury paradigms, cervical multilevel dorsal rhizotomy and midthoracic dorsal spinal cord hemisection. After dorsal root crush injury, the ROCKII(-/-) mice recovered use of the affected forepaw more quickly than did controls. Moreover, multiple classes of sensory axons regenerated across the dorsal root entry zone into the spinal cord of mice lacking ROCKII. After the spinal cord injury, ROCKII(-/-) mice showed enhanced local growth of raphespinal axons in the caudal spinal cord and corticospinal axons into the lesion site. Improved functional recovery was not observed by Basso Mouse Scale score following dorsal hemisection, likely due to developmental defects in the nervous system. Together, these findings demonstrate that the ROCKII gene product limits axonal growth after CNS trauma.

Localized hypertrophic neuropathy: a case report and review of the literature.

The case is presented of a 71-year-old man with a 6-year history of symptoms suggestive of carpal tunnel syndrome, which did not improve despite two surgical procedures. On further investigation, a fusiform enlargement of the median nerve was found above the elbow, which was found on biopsy to be localized hypertrophic neuropathy (LHN). This case is the first to be described affecting the median nerve. The literature regarding LHN is reviewed, with discussion of the differential diagnoses and possible etiology of this rare lesion.

Pathological findings in subsynovial connective tissue in idiopathic carpal tunnel syndrome.

Recent studies suggest that in patients with carpal tunnel syndrome, pathological changes occur in the subsynovial connective tissue. Such changes are non-inflammatory synovial fibrosis and vascular proliferation. Thickening of the tendon sheet may cause an increase of canal pressure and damages to the median nerve in the wrist; however, the causes of such events still remain to be clarified. We examined synovial specimens from 26 patients operated on for idiopathic carpal tunnel syndrome. Analysis included histological, ultrastructural and immunohistochemical examination in order to establish a pathological underlying pattern. An explanation for the pathogenesis of the found changes suggested. Our data confirm the presence of a non-inflammatory fibrosis with irregular bundles of collagen. De novo blood vessel formation was also noted. Interestingly the neo-angiogenesis consists of anomalous vessels and may be triggered from various cell types secreting vascular endothelial growth factor (VEGF), including macrophage-like elements similar to endothelial progenitor cells. Therefore, we believe that in the future a non-surgical management of carpal tunnel syndrome might be conjecturable via anti-VEGF drugs.

Lipofibromatous hamartoma of the median nerve in patients with macrodactyly: diagnosis and treatment of a rare disease causing carpal tunnel syndrome.

Lipofibromatous hamartoma is a very rare benign peripheral nerve tumour. It is mostly encountered in the proximal extremities of young adults, involving the median nerve in the majority of cases. We present two patients with macrodactyly and carpal tunnel syndrome caused by lipofibromatous hamartoma of the median nerve and discuss diagnosis and treatment of the disease. A 10-year-old girl with a congenital progressive macrodactyly of her right index finger presented with a slowly growing mass in her right palm and pain and numbness, along with motor and sensory deficits in the median nerve distribution. Treatment consisted of carpal tunnel release, epineurolysis and partial excision of the fibrofatty tissue. The second patient, a 25-year-old man presented with a swelling in his left palm and findings compatible with carpal tunnel syndrome. Intraoperatively, the lesion presented as sausage-shaped enlargement of the median nerve by fibrofatty tissue. After carpal tunnel release, a partial excision of the mass with epineurolysis was performed. In both patients, histology showed nerve bundles separated by abundant fibrofatty tissue. In the girl, a proliferation of dysplastic perineurial cells could be observed. The suspected diagnosis for patients with macrodactyly and clinical signs of carpal tunnel syndrome should be lipofibromatous hamartoma. A carefully taken history, physical examination, X-ray, and MRI are important for its correct diagnosis. The surgical management remains controversial. Treatment should include decompression of the median nerve at points of compression, partial excision of the fibrofatty tissue, and debulking of soft tissue. In some cases, an epineurolysis can be additionally performed.

Poly(ε-Caprolactone) Nanofiber Wrap Improves Nerve Regeneration and Functional Outcomes after Delayed Nerve Repair.

BACKGROUND: The purpose of this study was to assess the efficacy of biodegradable, electrospun poly(ε-caprolactone) nanofiber nerve conduits in improving nerve regeneration. METHODS: The authors used a rat forelimb chronic denervation model to assess the effects of poly(ε-caprolactone) conduits on improving nerve regeneration and upper extremity function. Three groups of rats were examined: (1) negative-control animals (n = 5), which underwent 8 weeks of median nerve chronic denervation injury followed by repair with no conduit; (2) experimental animals (n = 5), which underwent 8 weeks of median nerve chronic denervation followed by repair and poly(ε-caprolactone) nerve conduit wrapping of the nerve coaptation site; and (3) positive-control animals (n = 5), which were naive controls. All animals underwent compound muscle action potential and functional testing. At 14 weeks after repair, the median nerve and flexor muscles were harvested for histologic analysis. RESULTS: Histomorphometric analysis of regenerating median nerves demonstrated augmented axonal regeneration in experimental versus negative control animals (total axon count, 1769 ± 672 versus 1072 ± 123.80; p = 0.0468). With regard to functional recovery, experimental and negative-control animals (1.67 ± 0.04 versus 0.97 ± 0.39; p = 0.036) had regained 34.9 percent and 25.4 percent, respectively, of baseline hand grip strength at 14 weeks after repair. Lastly, less collagen deposition at the nerve coaptation site of experimental animals was found when compared to control animals (p < 0.05). CONCLUSION: Biodegradable, poly(ε-caprolactone) nanofiber nerve conduits can improve nerve regeneration and subsequent physiologic extremity function in the setting of delayed nerve repair by decreasing the scar burden at nerve coaptation sites.

[Acute carpal tunnel syndrome caused by a thrombosis of a persistent median artery: 2 case reports]. / Canal carpien aigu : penser à la thrombose de l'artère persistante du nerf médian !

Carpal tunnel syndrome is a common peripheral neuropathy, usually idiopathic or post-traumatic due to the compression of the median nerve. Numbness and paresthesias in the median nerve distribution are the most common symptoms associated with this condition. Persistent median artery is a rare anatomic variation, thrombosis of this additional artery can be responsible for an acute carpal tunnel syndrome, and patients frequently complain about coldness and acute hand swelling. These unusual features must lead clinicians to think of a vascular cause. The diagnosis can be easily confirmed by using ultrasound doppler, but CT-scan and MRI are sometimes helpful. We describe 2 cases of acute carpal tunnel syndrome due to thrombosed persistent median artery, including a case of thromboangiitis obliterans. These thrombosis might also be due to traumatic causes. No guidelines are currently available to help physicians for the management of carpal tunnel syndrome from thrombosed persistent median artery. Antiplatelet therapy, statin, anticoagulant might be helpful, and surgery has sometimes be reported as effective.

Glomus tumor and glomangioma of the nerve. Report of two cases.

As a rule, normal human nerve does not contain glomus bodies. Nonetheless, rare examples of glomus tumors do arise in peripheral nerves of various sizes. Their pathobiological characteristics are poorly understood, but reported examples have been small and clinically benign. The authors identified in 1 patient each a glomus tumor and a glomangioma involving nerve. Clinical histories as well as imaging and surgical findings were reviewed. All available H & E-stained slides were examined in both cases. Immunohistochemical stains and electron microscopy, as appropriate, were also performed. The lesions were subtotally and completely resected, respectively. An uneventful postoperative recovery was noted in both patients. Glomus tumors and glomangiomas can involve major nerves on rare occasions. They seem to follow a favorable clinical course, and conservative resection can be of benefit.