Chronic sensorimotor polyneuropathy associated with tacrolimus immunosuppression in renal transplant patients: case reports

Tacrolimus is used widely for immunosuppression following transplantation. It has rarely been linked to the development of peripheral neuropathy. However, one study also reported improvement in peripheral neuropathy after tacrolimus use. We have reported herein two patients who developed chronic sensorimotor polyneuropathy after tacrolimus use following renal transplantation. One patient had an unusual presentation with bilateral facial and extremity weakness and a relapsing course. The other patient presented with focal sensory symptoms in one hand. Electrophysiological studies confirmed widespread, predominantly demyelinating or axonal polyneuropathy. We concluded that patients on tacrolimus should be carefully monitored for symptoms suggestive of peripheral neuropathy.     

Hypertrophic neuropathy of the cauda equina: case report

OBJECTIVE AND IMPORTANCE: Hypertrophic neuropathy of the cauda equina (HNCE) is a rare form of peripheral neuropathy. The diagnosis is complicated by an insidious clinical presentation and complex radiographic images. We present a case of HNCE caused by chronic inflammatory demyelinating polyneuropathy with symptomatic improvement after decompressive lumbar laminectomy and dural expansion. CLINICAL PRESENTATION: A 54-year-old woman with a history of back pain since she was in her 20s presented with low back and radicular pain that had increased during a period of 6 months, bilateral lower-extremity weakness, and sensory loss in the right thigh. Magnetic resonance imaging of the lumbosacral spine revealed multiple, poorly enhancing mass lesions and apparent intrathecal nerve root thickening from L1 to L5. INTERVENTION: An L1-L5 decompressive laminectomy, performed with continuous somatosensory evoked potential and electromyographic monitoring, revealed multiple segmentally enlarged nerve roots. One nerve root that did not respond to high levels of stimulation was identified. This root was resected and submitted for pathological analysis. The dura was expanded with an 11-cm-long dural patch. The pathological examination revealed hypertrophic neuropathy, with extensive S-100-positive "onion bulb" formation. The patient's symptoms improved postoperatively. CONCLUSION: HNCE is a rare disorder that can cause radicular pain and lower-extremity weakness, sensory loss, and hyporeflexia. One possible cause is demyelinating polyneuropathy. Although medical management is typically effective in the treatment of demyelinating polyneuropathy, it has little effect on compressive symptoms caused by intradural nerve root enlargement. As this case demonstrates, surgical management of symptomatic radiculopathy by lumbar laminectomy is a reasonable and effective approach to the treatment of HNCE.     

Autonomic dysfunction in acute inflammatory demyelinating polyneuropathy

Acute inflammatory demyelinating polyneuropathy (AIDP) is an autoimmune process that is characterized by progressive weakness, mild sensory changes and autonomic dysfunction. It is a rare disorder, afflicting about 1 person in 100,000. Yet, since the decline in the number of polio cases, it represents the most common cause of acute neuromuscular paralysis. AIDP is thought to arise as a result of the production of antibody to bacterial Campylobacter jejuni, sugar-containing surface antigen(s) that, due to molecular mimicry, cross-react with the myelin sheath and the axons of nerve cells. Antibody and/or cell mediated immune reactions are believed to produce degeneration of the nerve or interruption of neurotransmission. Autonomic dysfunction include: transient hypertension or, less often, hypotension, sinus tachycardia, bradycardia, urinary retention who usually improves in parallel with motor and sensory function. Our purpose was to study the disturbances of autonomic function of the patients with acute idiopathic demyelinating polyneuropathy. We had studied 36 patients with AIDP admitted in I-st Clinic of Neurology, Iassy, between 1998 and 2002. Analyzing the evolution of these autonomic disturbances we observed the early onset together with motor symptoms, tachycardia and tachypnea was more persistent and a real vital risk factor.     

Paraproteinemia Associated with Demyelinating Polyneuropathy or Myositis: Treatment with Plasmapheresis and Immunosuppressive Drugs

Abstract: Four patients with chronic, progressive demyelinating peripheral polyneuropathy were found to have paraproteinemia. Two of the patients had multiple myeloma [both immunoglobulin (Ig)G lambda]. The other two had benign gammopathies: one of IgG kappa type and the other one with marked polyclonal elevation of IgM. Immunofluorescence studies revealed deposits of the abnormal serum immunoglobulin along the myelin sheaths in two of the patients in whom sural nerve biopsies were performed. All four patients were treated with plasmapheresis in combination with immunosuppressive drugs. Favorable responses to the therapy was observed in all four patients, but the degree of response varied from patient to patient. Two patients who presented clinical and electromyographic findings consistent with polymyositis were found to have serum IgG kappa M components. Immunofluorescence studies performed on muscle biopsy material from both patients revealed deposits of the abnormal serum paraprotein along the sarcolemmal basement membrane. A treatment course of plasmapheresis and immunosuppressive drugs resulted in a sustained increase of muscle strength in both patients.     

Hypertrophic neuropathy of the cauda equina

A 72-year-old woman with a history of lower extremity weakness, pain, and sensory loss in the right thigh for the last 15 months had deteriorated during a period of 6 months. Magnetic resonance imaging of the lumbosacral spine revealed apparent nerve root thickening from L1 to S and spinal canal stenosis. An L1–L3 decompressive laminectomy and durotomy revealed multiple segmentally enlarged and congestive nerve roots. A 1-mm segment of nerve root was resected and submitted for pathological analysis. The pathological examination revealed hypertrophic neuropathy, with nonspecific inflammation. After steroid use, the patient’s symptoms improved. HNCE is a rare disorder that can cause pain and lower extremity weakness, sensory loss, and hyporeflexia. One possible cause is chronic inflammatory demyelinating polyneuropathy (CIDP), but the intrathecal nerve roots are enlarged only patchily and canal stenosis is also present, so the diagnosis is unclear. As this case demonstrates, surgical management and administration of steroids are an effective treatment for some HNCE.     

Peripheral neuropathy in HIV patients: treatment by decompression of peripheral nerves

Peripheral neuropathy is the most common nerve disorder in human immunodeficiency virus (HIV) patients. Distal symmetrical sensory polyneuropathy (DSP) affects roughly one third of HIV patients. With the introduction of antiretrovirals, more patients are surviving longer, and chronic complications are surfacing. Three consecutive patients with at least a 5-year history of HIV presented during the period from 2007 to 2009. All three patients were on antiretrovirals and had no other comorbid conditions such as spinal pathology or diabetes. All patients had symptoms of pain, numbness, and weakness. Quantitative sensory testing and/or electromyography/nerve conduction testing (EMG/NCT) were performed preoperatively and correlated with the presence of Tinel signs. Targeted nerve releases were performed in four extremities, for a total of 18 nerves. All three patients had symptomatic improvement at 1 year with a visual analog scale (VAS) reduction in pain by at least five points. Quantitative sensory testing showed improvement, as did two EMG/NCTs obtained postoperatively. This showed improvement in conduction velocity at the fibular tunnel and posterior tibial nerve at the tarsal tunnel. This is the first report of nerve decompressions in the lower and upper extremity of HIV patients in the literature outside of the median nerve in the carpal tunnel.     

Charcot-Marie-Tooth disease: peripartum management of two contrasting clinical cases

Charcot-Marie-Tooth disease is a rare hereditary motor and sensory demyelinating polyneuropathy with potentially severe and debilitating peripheral symptoms. Respiratory muscles and vertebral anatomy can be affected, both of which may have significant impact on any planned or unplanned anaesthetic intervention during labour. We describe two cases at opposite ends of a wide spectrum of disease severity. The first case illustrates an approach to the management of a mildly affected patient who was permitted to labour normally, but nevertheless needed a detailed antenatal plan in order to allay her anxiety and prepare for potentially complicated labour analgesia and operative delivery. Spontaneous delivery of a healthy infant did not require anaesthetic intervention during labour. The second woman had severe scoliosis and marked respiratory impairment and required non-invasive ventilatory support for one week before scheduled caesarean section. A single-shot spinal anaesthetic was used as a spinal catheter could not be sited. This produced a high block for which a brief period of respiratory assistance was required. Perioperative management and subsequent high dependency care are discussed.     

Peroneal nerve palsy due to rare reasons: a report of three cases

Peroneal nerve entrapment was diagnosed in three patients (2 males, 1 female) by clinical and electrophysiological studies. Of these, one patient had postural bilateral involvement due to prolonged squatting, while two patients had mechanically-induced entrapment. Initially, all the patients were treated conservatively with a drop-foot splint and vitamin B. One patient responded to treatment; in one patient with bilateral involvement, right-sided peroneal nerve palsy improved. Upon detection of no clinical and electrophysiological improvement after three months of conservative treatment, surgical decompression was performed in two patients, which resulted in a successful outcome in the patient with bilateral palsy. Incomplete recovery was obtained in the other patient with diabetic polyneuropathy.     

Evaluation of spatio-temporal gait parameters and gait symmetry in diabetic polyneuropathic patients with burn injury: A pilot study

PurposeThe purpose of this study was to investigate the effects of burn injury on spatio-temporal gait parameters and gait symmetry in individuals with diabetic polyneuropathy (DPN)-related lower extremity burn injury.MethodsDemographic information and the physical examinations of the lower extremities of 14 patients with unilateral lower extremity burn injury due to DPN (DPN_B) and 14 uninjured patients with DPN (DPN₀) were recorded. The GAITRite computerized gait analysis system was used to evaluate the spatio-temporal parameters of gait. Symmetry Index (SI) was calculated to determine gait symmetry. The Mann Whitney U test was used to determine the demographical and clinical differences between the groups, the Wilcoxon’s test was used to compare both sides of all the participants for all gait parameters and linear regression analysis was used to find the variables that affect gait parameters.ResultsThe groups were similar in terms of their demographic information except for age. Compared with the DPN₀ group, DPN_B group had increased extent of area with sensory loss and severity of DPN, decreased ankle joint range of motions and intrinsic foot muscles strength and they displayed lower gait speed, step length, stride length and swing percentage but their double support and stance percentage were increased.ConclusionThis study results have shown that, spatio-temporal gait parameters of DPN_B patients are different. These differences in spatiotemporal parameters are found on both sides, probably owing to the symmetric and bilateral sensory loss is superior to unilateral burn injury due to symmetrical and bilateral sensory loss. Gait analysis should be considered in order to reveal the changes in gait parameters and to improve appropriate healing process of patients with DPN-related lower extremity burn injury.     

Failure of relaxometry in diabetic polyneuropathy]

Disturbances of the peripheral nervous system, summarised under the term "diabetic polyneuropathy", are a well-known complication of long-term diabetes mellitus. However, there is little information about the fact that these conditions may lead to difficulties in monitoring neuromuscular blockade by peripheral nerve stimulation during general anaesthesia. We report two diabetic patients (40 resp. 62 years of age) in whom a total of four ophthalmological operations were performed under general anaesthesia. In all cases monitoring of neuromuscular transmission was attempted by stimulation of the left ulnar nerve slightly proximal to the wrist, but not even the usual calibration could be obtained in either patient. Checks confirmed that the nerve stimulator was functional and the electrodes--in all cases self-adhesive, pregelled surface electrodes--were placed correctly. In order to evaluate the hypothesis that disturbances of peripheral nerve function might be responsible, the patients were examined neurophysiologically in the postoperative period. Both patients showed severe alterations in peripheral nerve conduction: a sensory response could not be evoked in any of the extremities and the ulnar distal motor latency time was pathologically prolonged (5.4 ms in patient 1 and 4.9 ms in patient 2; normal: 1.4-4.0 ms). Therefore, in accordance with the clinical symptoms, the diagnosis of diabetic polyneuropathy was established. These cases demonstrate that severe disturbances of the peripheral nervous system may render neuromuscular monitoring impossible.     

Sciatic nerve release following fracture or reconstructive surgery of the acetabulum

BACKGROUND: Sciatic neuropathy associated with acetabular fractures can result in disabling long-term symptoms. The purpose of this retrospective study was to evaluate the effect of sciatic nerve release on sciatic neuropathy associated with acetabular fractures and reconstructive acetabular surgery. METHODS: Between 2000 and 2004, ten patients with sciatic neuropathy associated with an acetabular fracture were treated with release of the sciatic nerve from scar tissue and heterotopic bone. Additional surgical procedures included open reduction and internal fixation of the acetabulum (five patients), removal of hardware and total hip arthroplasty (three patients), and removal of hardware alone (one patient). The average age of the patients was forty-three years. All patients were followed with serial examinations and assessments for a minimum of one year (average, twenty-six months). RESULTS: All patients had partial to complete relief of radicular pain, of diminished sensation, and of paresthesias after the nerve release. Four of seven patients with motor loss and two of five patients with a footdrop demonstrated improvement in function after the nerve release. No patient had evidence of worsening on neurologic examination after the release. CONCLUSIONS: Sciatic nerve release during reconstructive acetabular surgery can decrease the sensory symptoms of preoperative sciatic neuropathy associated with a previous acetabular fracture. Motor symptoms, however, are less likely to resolve following nerve release.     

“Silent” bone stress injuries in the feet of diabetic patients with polyneuropathy: a report on 12 cases

Background Bone stress injuries are rarely being diagnosed in patients with sensory neuropathy, most likely because they may be silent in terms of pain. Load-related pain is considered a key feature of any bone stress injury, a symptom, which may be partially or completely absent in subjects with sensory neuropathy (loss of protective sensation). We evaluated the clinical course of bone stress injuries in insensitive feet in diabetic patients with polyneuropathy. Methods We investigated 12 consecutive diabetic patients with bone stress injuries of the foot (bone marrow edema, bone bruise and microtrabecular fractures, on magnetic resonance imaging MRI), which were undetectable on plain X-ray. All patients suffered from diabetic polyneuropathy, none of them had an active foot ulcer. Results The patients presented with a swollen foot, which was only mildly painful and did not prevent them from walking. Complaints were related to the swelling, which increased during load-bearing. In seven cases, a traumatic event preceding the onset of symptoms could be ascertained. MRI disclosed stress injuries in 2.5 (1–8) [median (range)] bones per foot. In 11 patients, treatment was started immediately by off-loading with total contact cast for 17 (8–52) weeks, followed by gradual increase in weight bearing. One patient unfortunately received off-loading treatment only after deforming fractures had developed. All bone injuries healed uneventuelly in eight patients, and with residual mild osteoarthrosis in three patients without gross deformities. However, the untreated patient developed severe Charcot foot deformity. Conclusions In diabetic patients with polyneuropathy, symptoms of bone stress injuries of the foot are atypical, in that there is load-related swelling rather than load-related pain. Immediate diagnosis, and treatment with off-loading, leads to a restitutio ad integrum like in non-neuropathic patients. Delayed cessation of overuse, however, may cause irreversible joint and bone damage (Charcot foot).     

Outcomes of cubital tunnel surgery among patients with absent sensory nerve conduction

OBJECTIVE: To report the outcomes of cubital tunnel surgery for patients with absent ulnar sensory nerve conduction. METHODS: The charts of 34 patients who exhibited clinical symptoms of ulnar nerve entrapment at the elbow and who had electromyography-confirmed prolonged motor nerve conduction across the cubital tunnel in association with absent sensory nerve conduction were reviewed. The mean age was 63 years, and the mean symptom duration was 17 months. Four patients had bilateral symptoms. Surgery was performed for 38 limbs, i.e., neurolysis for 21 limbs and subcutaneous transposition for 17 limbs. Fifteen limbs demonstrated associated ulnar nerve-related motor weakness. The mean postoperative follow-up period was 4 years (range, 3 mo to 11 yr). RESULTS: Sensory symptoms (i.e., pain, paresthesia, and two-point discrimination) improved in 20 limbs (53%), and muscle strength improved in 2 limbs (13%). Improvements in sensory symptoms were not related to patient age, symptom duration, cause, severity of prolonged motor nerve conduction, select psychological factors, associated medical diseases, associated cervical pathological conditions, or type of surgery. Improvements in sensory symptoms were significantly decreased among patients who had experienced cervical disease for more than 1 year and patients with bilateral symptoms. CONCLUSION: Patients with cubital tunnel syndrome who have absent sensory nerve conduction seem to experience less improvement of sensory symptoms after surgery, compared with all patients with cubital tunnel syndrome described in the literature. Bilateral symptoms and delayed surgery secondary to associated cervical spine disease seem to be significant negative factors for postoperative improvement of sensory symptoms. Sensory symptoms improved similarly among patients who underwent neurolysis or subcutaneous transposition     

Neuropathic arthropathy of the elbow. A report of five cases.

BACKGROUND: Neuropathic arthropathy of the elbow is rare and characterized by a painless but unstable articulation. The functional capacity of patients with this condition has not been reviewed in detail. METHODS: Five male patients, with an average age of fifty-one years, were treated for neuropathic arthropathy of the elbow. The underlying conditions associated with the arthropathy included syringomyelia, insulin-dependent diabetes mellitus, end-stage renal failure, and two cases of polyneuropathy of unknown cause. Four patients sought medical attention after a specific traumatic event. Peripheral sensory and motor dysfunction was present in each patient. Radiographs of the elbow revealed dislocation, fracture fragmentation, and heterotopic ossification. Our management of the neuropathic elbows centered on maintenance of a functional arc of motion through physical therapy aimed at regaining muscle strength and the use of orthoses for support. Operative treatment was performed for an associated ulnar or radial nerve compression syndrome in three patients, and an open reduction and internal fixation of an unstable proximal ulnar nonunion associated with loose implants was performed in one. RESULTS: The patients were followed for an average of nineteen months, with a range of twelve to thirty-six months. All patients had a pain-free elbow with a functional range of motion at the most recent follow-up examination, and none wished to have further treatment. The operatively treated ulnar nonunion united successfully. All three patients treated surgically for an associated nerve compression syndrome had recovery of nerve function. CONCLUSION: In the face of instability and gross distortion of the joint, the patients in this series demonstrated remarkably good function.     

Ultrasound-guided peripheral regional blockade in patients with Charcot-Marie-Tooth disease: a review of three cases

PURPOSE: To describe the clinical presentation of three patients with Charcot-Marie-Tooth disease, who underwent uneventful upper limb surgery following successful peripheral nerve blockade, and to review the anesthetic implications in patients with Charcot-Marie-Tooth disease.Clinical features: In three patients with Charcot-Marie-Tooth disease presenting for surgery of the upper limb, the motor response, following nerve stimulation, was suboptimal. However, ultrasound guidance was effective in visualizing the needle-nerve interaction, and local anesthetic was injected around the nerves. Good block ensued and surgery proceeded in all patients without complications. No exacerbation of the neurological condition was observed in any patient.Conclusions: Charcot-Marie-Tooth disease is a demyelinating, hereditary, motor and sensory neuropathy characterized by abnormalities of nerve conduction. Regional anesthesia of the upper limb is feasible in these patients, and these cases show that ultrasound guidance makes peripheral nerve block possible in patients for whom traditional methods of nerve localization fail.     

Medial humeral condyle fractures in adolescents: treatment and complications

The very rare isolated medial humeral condyle fractures in children and adolescents pose significant problems to orthopedic surgeons such as ulnar nerve injury, trochlear irregularities, fishtail deformity and motion loss. Six adolescent boys with displaced medial humeral condyle fractures with an average age of 14.8 (range 12–17) were included in this study. Patients were followed-up with a mean of 39.8 months (range 21–72). In three patients, there were clinical signs of ulnar nerve dysfunction at initial presentation. Of these, two had only mild hypoesthesia on the ulnar side. The other patient had sensory and motor loss of ulnar nerve at initial presentation complicated by hypothenar atrophy and anhydrosis during follow-up. Complete recovery was seen in this patient only at 2 years. One patient without a neurologic compliant at initial presentation developed mild hypoesthesia on the ulnarly innervated areas postoperatively with complete resolution. Medial skin incision was used for open reduction and internal fixation. No signs of gross morphologic damage were seen on the nerve. The symptoms resolved postoperatively in 3 months time in three of the patients, but in the remaining one, it took 2 years for complete resolution of the symptoms. Also, trochlear irregularity and fishtail deformity were seen on radiographs of two different patients. In all but one patients, motion loss ranging from 10° to 20° was evident on physical examination. The most important finding of this study revealed that transient ulnar nerve dysfunction was seen two-thirds of the patients in this series than any other series reported previously. In three series, only one case of ulnar nerve injury was reported from a total of 19 patients. A possible explanation for such a difference may be the older age in this series.     

Peripheral nerve function in patients with chronic bronchitis receiving almitrine or placebo.

A double blind prospective study of the effect of almitrine bismesylate and placebo on peripheral-nerve function was carried out in 12 patients with chronic bronchitis and arterial hypoxaemia (mean (SD) FEV1% predicted 38 (16), arterial oxygen tension (PaO2) 7.56 (0.76) kPa). Of the seven patients who took placebo, none developed symptoms or signs of peripheral neuropathy. One patient who had abnormal lower limb sensory nerve conduction initially showed improvement of sensory conduction but deterioration in motor conduction during the 12 month study period. Two further patients developed some slowing of motor conduction velocities in their right lateral popliteal nerve. Five patients received almitrine and all showed an improvement in PaO2 (mean from 7.0 to 7.9 kPa). None had symptoms or signs of peripheral neuropathy on entry to the study; one patient had evidence of impaired nerve conduction on electrophysiological testing. Three patients developed symptoms and signs of peripheral neuropathy during the 12 months of the study and a fourth developed peripheral neuropathy at 18 months, having continued to receive almitrine. Studies of nerve physiology showed abnormalities in the lower limbs of all four patients. Recovery was poor, possibly because of the long half life of almitrine. The studies suggest that almitrine may precipitate peripheral neuropathy in patients with chronic obstructive pulmonary disease. Patients should be warned of this potential complication so that the drug can be stopped as soon as symptoms develop.     

The use of sacral neuromodulation for the treatment of non-obstructive urinary retention secondary to Guillain-Barré syndrome

Guillain-Barré syndrome, an acute autoimmune polyneuropathy and demyelinating disease, is characterized by weakness, sensory loss, areflexia, pain, autonomic dysfunction, and occasionally, micturition disturbances including voiding difficulty, urinary retention, nocturnal urinary frequency, and urge incontinence. Typically, urinary dysfunction resolves simultaneously with other neurologic deficits. We report the case of a 20-year-old woman with Guillain-Barré syndrome and persistent urinary retention 18 months following initial diagnosis. This patient is the first described in the literature to undergo successful treatment with sacral neuromodulation. Immediately following neuromodulator placement, the patient voided spontaneously and has had no voiding dysfunction or postvoid residual after 5 months of follow-up.     

Long-term consequences of domino liver transplantation using familial amyloidotic polyneuropathy grafts

Domino liver transplantation (DLT) using grafts from patients with familial amyloidotic polyneuropathy (FAP) is an established procedure at many transplantation centers. However, data evaluating the long-term outcome of DLT are limited. The aim of the present study was to analyze the risk of de novo polyneuropathy, possibly because of amyloidosis, and the patient survival after DLT. At our department, 28 DLT using FAP grafts were conducted between January 1997 and December 2005. One patient was twice subjected to DLT. Postoperative neurological monitoring of peripheral nerve function was performed with electroneurography (ENeG) in 20 cases. An ENeG index based on 12 parameters was calculated and correlated to age and/or height. Three patients developed ENeG signs of polyneuropathy 2-5 years after the DLT, but with no clinical symptoms. The 1-, 3- and 5-year actuarial patient survival in hepatocellular carcinoma (HCC) patients (n = 12) and non-HCC patients (n = 15) was 67%, 15%, 15% and 93%, 93%, 80%, respectively (P = 0.001). Development of impaired nerve conduction in a proportion of patients may indicate that de novo amyloidosis occurs earlier than previously expected. Survival after DLT was excellent except in patients with advanced HCC.     

Treatment of Chronic Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated, non–length-dependent polyradiculoneuropathy that is progressive or relapsing over a period of at least 8 weeks, often evolving over time to a relatively symmetric pattern. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral reaction to the peripheral nerve myelin sheath involving nerve roots and proximal and distal nerves. Early medical treatment of CIDP is important to prevent axonal loss occurring as a secondary effect of progressive demyelination. Only three treatments for CIDP have demonstrated benefit in randomized controlled studies: corticosteroids, plasma exchange, and intravenous immunoglobulin. About 25% of patients fail to respond to these treatments or respond inadequately. These treatments have similar efficacy but differ significantly in cost and adverse effects. These factors are considered in treatment selection.