Nerve Decompression in the Lower Leg Results in an Improvement in Symptoms in Patients With Both Diabetic and Idiopathic Polyneuropathy.

BACKGROUND: Patients suffering from polyneuropathy often complain of pain, tingling, and numbness sensations, as well as an increased risk of falling with the corresponding subsequent complications. If symptoms persist after conservative treatment options have been exhausted, nerve decompression in the lower extremity, as described by Dellon, can bring about an improvement in symptoms in many patients. Dellon originally reported that this surgery led to very successful outcomes in patients with diabetic polyneuropathy. In this study, we compare our postsurgical results in patients with diabetic versus idiopathic polyneuropathy. METHODS: Thirty-three patients with idiopathic or diabetic polyneuropathy who had undergone Dellon nerve decompression in the lower extremity between 2011 and 2013 were included in the retrospective study. Pain (numeric rating scale [NRS] 0-10; 0, no pain; 10, worst imaginable pain), tingling, numbness, Hoffmann-Tinel sign, and Semes-Weinstein monofilament were assessed in 20 patients with diabetic polyneuropathy and in 13 patients with idiopathic polyneuropathy. RESULTS: Three months after surgery, a significant reduction in pain was evident in patients with diabetic polyneuropathy, from a preoperative level of NRS 4.9 (minimum, 0; maximum, 10) to 2 (minimum, 0; maximum, 8; P = 0.005). Ninety percent of patients complained of tingling ( P = 0.000) before surgery and 18% after surgery, whereas 100% complained of numbness before surgery and 41% ( P = 0.000) after surgery. One hundred percent of patients had no measurable surface sensitivity before surgery (measured with the Semes-Weinstein monofilament), whereas 3 months after surgery, only 24% of patients still had no measurable surface sensitivity ( P = 0.000). A positive Hoffmann-Tinel sign was recorded in 85% of patients before surgery and only in 11% 3 months after surgery ( P = 0.000). In the case of patients with idiopathic polyneuropathy, a reduction in pain was evident 3 months after surgery, from a preoperative level of NRS 3.9 (minimum, 0; maximum, 9) to 2.2 (minimum, 0; maximum, 9; P = 0.058). Seventy-seven percent of patients complained of tingling before surgery and 42% after surgery ( P = 0.111), whereas 92% complained of numbness before surgery and 50% after surgery ( P = 0.030). Seventy-seven percent of patients had no measurable surface sensitivity before surgery (measured with the Semes-Weinstein monofilament), whereas 3 months after surgery, only 33% of patients still had no measurable surface sensitivity ( P = 0.047). A positive Hoffmann-Tinel sign was recorded in 62% of patients before surgery and only in 17% 3 months after surgery ( P = 0.041). CONCLUSIONS: Not only patients with diabetic polyneuropathy but also those with idiopathic polyneuropathy benefit from Dellon nerve decompression surgery in the lower extremities.

Saphenous nerve to posterior tibial nerve transfer: A new approach to restore sensations of sole in diabetic sensory polyneuropathy.

BACKGROUND: Loss of sensations in the sole following diabetic sensorimotor polyneuropathy (DSPN) leads to diabetic foot ulcers and its sequelae. We hypothesized that sensory reinnervation of sole by transfer of saphenous nerve (SN) to sensory fascicles of posterior tibial nerve (PTN) in these patients may reverse the neuropathy. METHODS: This prospective interventional case series included patients with advanced DSPN and intact sensory supply of SN. PTN was neurotized by transfer of SN nerve in the tarsal tunnel and postoperatively sensations of the sole were tested. Any existing ulcers on sole were noted and their healing was monitored. FINDING: A total of 17 patients (22 feet), 9 male and 8 female, were included. Seven patients had ulcers in the feet. At 6 months follow-up all patients developed protective sensation in the sole. The average 2 PD improved from 60 mm to 45.5 mm, average vibration perception improved from 34.12 V to 24.33, Medical Research Council (MRC) score improved from S0 in 12 feet and S1 in 10 feet to S3+ in 13 feet, S3 in 5 feet, and S2 in 2 feet at 6 months along with healing of ulcers in all 7 feet. INTERPRETATION: Transfer of SN to PTN for sensory neurotization is an innovative and simple option to prevent complications of DSPN. This procedure has the potential to change the natural history of DSPN.

Spinal Cord Stimulation as a Treatment Option for Refractory Chemotherapy-Induced Peripheral Neuropathy: Case Report

Colorectal cancer is one of the most common oncological diseases. Chemotherapy is usually recommended as an adjuvant treatment for stage-II, -III, and -IV tumors. Approximately 10% of the patients develop neuropathic pain after chemotherapy, and they may remain refractory despite the administration of drugs that are commonly used to treat neuropathic pain. Spinal cord stimulation is a good treatment option for neuropathic pain of the lower limbs, and it should be trialed in patients with chemotherapy-induced peripheral neuropathy. We report the case of a patient with oxaliplatin-induced neuropathy and neuropathic pain refractory to oral medication who was successfully treated by spinal cord stimulation.

A 5-Year Follow-Up of The Benefits of an Exercise Training Program in Liver Recipients Transplanted Due to Familial Amyloidotic Polyneuropathy.

BACKGROUND: Supervised (SE) and home-based exercise (HBE) training regimes are effective on reconditioning patients with familial amyloidotic polyneuropathy (FAP) after liver transplantation, but research of the long-term retention of the benefits attained in patients with FAP has not yet been conducted. PURPOSE: In this 5-year follow-up study, we aimed to determine whether the exercise training gains in body composition, physical activity, and function promoted by a 24-week SE or HBE training regimes are retained in patients with FAP who resume normal activity. METHODOLOGY: Sixteen liver-transplanted patients with FAP were reassessed for body composition (dual X-ray absorptiometry), physical activity (questionnaire), and function (handgrip strength and 6-minute walk test). RESULTS: Total body fat increased with both exercise regimes during follow-up ( P < .05; η2 = 0.432-0.625) as well as femoral neck bone density ( P = .048; η2 = 0.119). However, gains in upper limbs muscle quality during follow-up ( P < .001; η2 = 0.597) were only found in the SE group ( P = .042; η2 = 0.245). Both exercise regimes showed retaining aptitudes in walking capacity ( P < .05; η2 = 0.329-0.460) and muscle mass ( P = .05; η2 = 0.245). Still, none could retain the physical activity levels. CONCLUSION: Long-term resumption of normal activity following a 24-week SE or HBE regime in patients with FAP resulted in loss of exercise induced increases in physical activity but counterweighted postoperative losses in bone mineral density and substantially retained the benefits in walking capacity, muscle mass, and quality, in particular, in the SE group.

Postoperative Physical Therapy Management of Tendon Transfer for Digital/Wrist Extension Due to Multifocal Motor Neuropathy.

Study Design Case report. Background Multifocal motor neuropathy is a progressive motor nerve disorder characterized by muscle weakness in the extremities. Muscle imbalance and weakness can become so severe that the involved extremity can be rendered nonfunctional. The purpose of this case report is to describe the physical therapy postoperative management of a patient who underwent a multiple tendon transfer to correct the loss of digital/wrist extension of the right upper extremity. Case Description A 38-year-old woman with a medical diagnosis of multifocal motor neuropathy, which caused muscle imbalance and weakness in the right hand, underwent a multiple tendon transfer to correct the loss of digit and wrist extension. The pronator teres was transferred and attached to the extensor carpi radialis longus and brevis. The palmaris longus was transferred and attached to the extensor pollicis longus. The flexor carpi radialis was transferred and attached to the extensor digitorum communis. The patient underwent static and dynamic splinting and a modified tendon transfer protocol starting at 3 weeks and ending at 16 weeks postsurgery. The patient attended therapy 1 to 3 times a week, depending on protocol stage and need for skilled therapy intervention. Outcomes Patient-reported outcome measures included the Disabilities of the Arm, Shoulder and Hand (DASH) survey to monitor the return of function and the numeric pain-rating scale to assess pain. At the initial evaluation (3 weeks postsurgery), the patient's DASH score was 87.5 and her pain score was 7/10. At discharge (16 weeks postsurgery), the patient's DASH score was 37.5 and her pain score was 0/10. Strength impairment was monitored with hydraulic hand dynamometers and manual muscle testing. At discharge, her hand grip strength was 4.5 kg, her key pinch strength was 4.1 kg, and her 3-jaw pinch strength was 2.3 kg. Manual muscle testing grades were 5/5 for elbow extension/flexion, 4/5 for forearm pronation/supination, 2/5 for wrist extension, 1/5 for wrist radial deviation, 4-/5 for wrist ulnar deviation, 3/5 for extension from digits 2 through 4, 3+/5 for thumb extension, 5/5 for wrist flexion, and 5/5 for flexion from digits 1 through 5. At 1 year postsurgery, the DASH survey was sent to the patient for completion, at which time she reported a DASH score of 24.17. Conclusion This case demonstrated good outcomes for a patient who underwent a multiple tendon transfer to correct digital/wrist extension loss caused by multifocal motor neuropathy. This report provides guidance on the postoperative management of a fairly rare neurological disorder with an established orthopaedic surgery. Level of Evidence Therapy, level 5. J Orthop Sports Phys Ther 2016;46(12):1071-1079. Epub 30 Oct 2016. doi:10.2519/jospt.2016.6707.

Long-term outcome of patients with hereditary transthyretin V30M amyloidosis with polyneuropathy after liver transplantation.

BACKGROUND: Liver transplantation halts production of mutated transthyretin (TTR), and thus it is an accepted treatment, with improved survival, in patients with hereditary (familial) amyloidosis with polyneuropathy (FAP). However, the effects of transplantation on the clinical manifestations of FAP have not yet been adequately clarified. This study aimed to investigate whether liver transplantation would improve the long-term clinical manifestations in FAP patients who had undergone transplantations. PATIENTS AND METHODS: We assessed 29 non-transplant and 36 transplant FAP V30M patients using an FAP clinical scoring system. RESULTS: The total clinical score of the non-transplant group increased and was significantly correlated with FAP duration; that of the transplant group increased slowly after transplantation. In patients 5 years or more after FAP onset, the total clinical scores of the transplant group were significantly lower than those of the non-transplant group. In the same patients, scores for sensory, motor, autonomic and organ impairments of the transplant group were significantly lower than those of the non-transplant group. CONCLUSIONS: Liver transplantation had beneficial effects on FAP clinical manifestations in patients with FAP TTR V30M. Liver transplantation should therefore be considered as an effective treatment in the clinical management of patients with FAP TTR V30M.

Coexistence of a T118M PMP22 missense mutation and chromosome 17 (17p11.2-p12) deletion.

INTRODUCTION: We describe a 6-year-old girl with a T118M PMP22 mutation and heterozygous deletion of PMP22 on chromosome 17 (17p11.2-p12) resulting in a severe sensorimotor polyneuropathy. METHODS: This study is a case report in which the relevant mutations are described. RESULTS: Foot pain, cavovarus feet, tibialis anterior atrophy, absent reflexes, and inability to walk were found when the patient was age 6 years. Nerve conduction studies showed evidence of a sensorimotor polyneuropathy and compressive mononeuropathies of bilateral median nerves at the wrist and ulnar nerves at the elbow. Genetic testing revealed deletion of a PMP22 allele and T118M PMP22 mutation in the remaining allele. CONCLUSIONS: The severe sensorimotor polyneuropathy and hereditary neuropathy with liability to pressure palsies (HNPP) in this patient was likely a consequence of both decreased expression of PMP22 causing features consistent with HNPP and unopposed expression of the T118M mutant form of PMP22 that is relatively benign in the heterozygous state. The T118M mutant form of PMP22 can be disease-modifying in the appropriate circumstances.

Genetic expression profile of human liver grafts in ischemia-reperfusion injury: comparison of familial amyloidotic polyneuropathy and deceased-donor liver grafts.

This study aimed to compare the histologic and molecular gene expression at several surgical times (beginning of harvesting, T0; end of cold ischemia period, T1; and after reperfusion, T2) to characterize the ischemia-reperfusion injury (IRI) in deceased-donor liver grafts harvested from patients with familial amyloidotic polyneuropathy (FAP). For this purpose, 54 patients undergoing liver transplantation were studied and divided into 3 groups: deceased donor to cirrhotic recipient (group 1; n = 27), deceased donor to FAP recipient (group 2; n = 15), and FAP donor to cirrhotic recipient (group 3; n = 12). The main comparison was performed between a histologic score (Suzuki score, adding steatosis and neutrophil infiltration), and molecular gene expression of the following genes: interleukin (IL) 1ß, IL-6, E-selectin, Fas-ligand, granzyme B, heme oxygenase 1 (HO1), and nitric oxide synthetase (iNOS2A). We observed less neutrophil infiltration levels in group 3 in sample T0 (P = .0082), which was associated with gene expression of HO1 in the biopsies at T2 (P = .022). In group 3, the molecular expression of genes related to attenuated proinflammatory reaction during IRI, iNOS2A at T0 and HO1 at T2, was detected. We conclude that FAP liver grafts express differently the genes associated with an attenuated proinflammatory reaction, presenting less neutrophil infiltration at harvesting. These findings add more knowledge about the better short-term outcomes in patients receiving this type of liver graft.

Long-term outcomes and complications of trabeculectomy for secondary glaucoma in patients with familial amyloidotic polyneuropathy.

OBJECTIVE: Secondary glaucoma is a serious complication in patients with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP). We assessed the long-term outcomes and complications of trabeculectomy with mitomycin C (MMC) for secondary glaucoma associated with FAP. METHODS: Medical case records of Kumamoto University Hospital were retrospectively reviewed. Twenty-one eyes of 13 patients (10 with FAP ATTR Val30Met; 3 with FAP ATTR Tyr114Cys) underwent trabeculectomy with MMC and follow-up of at least 2 years. The primary outcome measure was Kaplan-Meier survival, with failure of this treatment being defined as an intraocular pressure (IOP) of ≤5 mm Hg or ≥22 mm Hg on two consecutive visits or as additional operations needed to reduce IOP. Secondary outcome measures included complications, bleb characteristics, and additional postoperative interventions required. RESULTS: The mean postoperative follow-up period was 5.7 years (range, 2.2-12.7 years). Kaplan-Meier analysis indicated probabilities of success of 0.76, 0.67, and 0.53 at 1, 2, and 3 years after operation, respectively. Significant complications included ocular decompression retinopathy in 7 eyes (33%) and bleb encapsulation in 10 eyes (48%). Twelve eyes (57%) needed additional surgery, such as bleb revision or trabeculectomy with MMC, to reduce IOP. CONCLUSIONS: Trabeculectomy with MMC may not be optimal for patients with FAP-related glaucoma and may have several significant complications.

[Ultrasonographic diagnosis of inflammatory neuropathies].

Ultrasonographic nerve enlargement has primarily been reported in patients with inflammatory neuropathies such as chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy, Guillain-Barre syndrome, vasculitic neuropathy and leprosy. Nerve ultrasonography is a promising diagnostic supportive tool for inflammatory neuropathies. The ultrasonographic findings that are currently useful are 1) nerve enlargement primarily suggests the existence of inflammatory or demyelinating neuropathies and 2) for patients with CIDP or demyelinating Charcot-Marie-Tooth disease, the pattern of nerve enlargement is noted, and this pattern is useful for discriminating between these diseases. More precise evidence of ultrasonographic findings for inflammatory neuropathies should be established in the future.

Restoration of prehensile function for motor paralysis in Hopkins syndrome: case report.

Hopkins syndrome is a rare cause of poliomyelitis-like paralysis affecting 1 or more extremities after an acute attack of asthma. The exact etiology of Hopkins syndrome is not known. A 4-year-old girl developed acute asthma followed by complete flaccid paralysis of the left upper extremity. She underwent staged reconstruction using the double free muscle transfer technique. Rigorous postoperative physiotherapy was carried out to achieve a good functional outcome. At recent follow-up, 27 months after the first procedure, the patient was able to effectively use the reconstructed hand for most daily activities. She had good control and could perform 2-handed activities. The selection of a suitable operative treatment and suitable donor nerves is critical, and there are no clear guidelines in the literature. The double free muscle transfer can be effectively employed in similar cases to restore grasping function.

Marked cardiomegaly in a patient with familial amyloidotic polyneuropathy after orthotopic liver transplantation: a case study.

Hepatocyte-derived mutant amyloidogenic transthyretin (ATTR) causes familial amyloidotic polyneuropathy (FAP), for which orthotopic liver transplantation is an established curative treatment. However, some patients with FAP have cardiac amyloidosis after transplantation. Here, we describe a man with an autonomic disorder diagnosed as FAP ATTR Val30Met and marked cardiomegaly after liver transplantation. He underwent orthotopic liver transplantation at 49 years of age and was prescribed prednisolone to prevent graft rejection. Two years later, autonomic dysfunction and severe heart failure gradually developed. He died suddenly at 59. The autopsy revealed marked cardiomegaly (heart weight: 1020 g). Histological and ultrastructural examinations demonstrated massive amyloid deposition and unusual myocardial hypertrophic injury associated with nuclear translocation of the glucocorticoid receptor (GR). No other FAP patients without heart failure showed GR nuclear translocation. GR is a nuclear transcription factor that leads to myocardial hypertrophy, and cumulative prednisolone doses may promote marked cardiomegaly and severe cardiac amyloidosis.

Effect of stem cell transplantation for B-cell malignancies on disease course of associated polyneuropathy.

B cell dyscrasias are often refractory to medical treatments, and hematological stem cell therapy (SCT) may be warranted. It is not clear whether an associated polyneuropathy may also profit from SCT. In exceptional cases SCT has been tried in patients with monoclonal gammopathy and progressive polyneuropathy refractory to medical treatments. In a cohort of 225 patients with monoclonal gammopathy and polyneuropathy, we selected the six patients who underwent SCT and retrospectively examined the effects of SCT on the disease course of the associated polyneuropathy. In all patients except one, the indication for SCT was hemato-oncological (multiple myeloma in 4 patients and primary AL amyloidosis in 1). The remaining patient had an IgG monoclonal gammopathy of undetermined significance and a progressive and painful polyneuropathy for which she was treated with SCT. SCT led to improvement of motor scores and autonomic symptoms in one patient; three patients experienced improvement of neuropathic pain or sensory deficits but showed further progression of weakness. One patient showed no improvement at all. One patient died within 100 days after SCT. In conclusion, SCT as a treatment of refractory hematological malignancy may occasionally have a positive effect on the associated progressive polyneuropathy, although the benefits are very limited and the treatment-related mortality is high.

Histopathological confirmation of polyneuropathy in 11 dogs with laryngeal paralysis.

Acquired laryngeal paralysis (LP) is an important cause of upper airway obstruction in dogs. We hypothesize that LP may be part of a generalized polyneuropathy complex. Electro-diagnostic studies were performed in six dogs, and histopathological studies of muscle and nerve biopsies were obtained from 11 dogs diagnosed with acquired LP. Abnormalities in electrodiagnostic procedures were consistent with a generalized polyneuropathy. Loss of large-caliber nerve fibers and axonal degeneration were identified in nerve biopsies, and neurogenic atrophy was observed in muscle specimens. Abnormalities in electrodiagnostic studies and histopathology provide evidence that LP may be part of a generalized polyneuropathy. Establishing a diagnosis of a more involved disease process is relevant for long-term prognosis.

A review of therapeutic nerve decompression for neuropathy in Hansen's disease with research suggestions.

Nerve decompression surgery for leprosy neuritis has a long history and large literature. New understanding of the high frequency of spontaneous recovery from nerve function impairment requires re-evaluation of the value of decompression in acute nerve dysfunction with strong evidence-based protocols. Several reports and theoretical considerations suggest research avenues that might offer hope for prevention of long-term complications and relief of impairment and disabilities.

Quality of life in patients with familial amyloidotic polyneuropathy long-term after liver transplantation.

Liver transplantation aims to halt the progression of the disease in patients with familial amyloidotic polyneuropathy (FAP) caused by hereditary transthyretin-related (ATTR) amyloidosis. Insight in health-related quality of life of these transplanted FAP-patients can be of help to optimize health care delivery. The aim of this cross-sectional study was to assess the health-related quality of life of patients with FAP long-term after transplantation. Nine patients with a post-transplant follow-up of 4 years or more were included in the study. During the annual checks, health-related quality of life was measured with the Short Form-36 (SF-36). Data were compared with non-FAP transplanted patients with the same duration of follow-up and with the normal Dutch population. Pre-transplant, all patients had signs of mild to moderate peripheral polyneuropathy. The results showed that in patients with FAP health-related quality of life was stable in the first 4 years after transplantation. The domain of physical well-being at 4 years after transplantation was significantly lower compared to non-FAP transplanted patients and control Dutch population. The domain of emotional well-being was comparable with non-FAP controls. However, on most health areas patients with FAP scored lower than the non-FAP transplanted patients and the Dutch controls. After four years, the three patients with FAP with longest follow-up (9-12 years) deteriorated in all health domains, except in self-perceived mental health. This study, including only a small number of patients with FAP, shows a relatively low health-related quality of life after liver transplantation, which may deteriorate further with longer follow-up.

Critical illness polyneuropathy. Regression following cardiac operation.

Critical illness polyneuropathy is an acute neuromuscular disorder of severely ill patients that may also occur in complicated postoperative heart surgery course. This case report should make this disease known also as a preoperative condition that may regress following a successful cardiac operation.

Early Marjolin's ulcer in Bureau-Barriere syndrome.

Bureau-Barrière syndrome (BBS) describes a rare destructive process affecting skin and bone resulting from an acquired unilateral or bilateral polyneuropathy. Clinically, it is characterized by a triad of symptoms, namely, acral ulcerations, polyneuropathy, and bone lesions. To date, the development of Marjolin's ulcer in the course of BBS has not been described to the authors' knowledge.