Recurrent idiopathic lumbosacral plexopathy

Recurrent idiopathic lumbosacral plexopathy has rarely been reported in the literature. The present report describes a 59-year-old man presenting with recurrent episodes of acute leg pain, followed by weakness. After each episode, symptoms progressed for several months before peaking. Thereafter, gradual recovery ensued. Electrodiagnostic studies revealed primarily a patchy pattern of denervation in the distribution of part of the lumbosacral plexus, sparing the paraspinal muscles. Extensive evaluations for an underlying cause were unrevealing. Thus, these episodes are suggestive of recurrent idiopathic lumbosacral plexopathy, and the present case is compared with previous cases reported in the literature.     

Intrapartum maternal lumbosacral plexopathy

There are many conflicting theories regarding the mechanism and prognosis of acute foot drop during labor. We report seven women who had arrested labor and foot drop. Six had short stature and one had a large newborn. All had weakness of ankle dorsiflexion, eversion, and inversion, and sensory loss in the L-5 dermatome. Superficial peroneal sensory nerve action potentials (SNAPs) were small or absent in six patients, and the sural SNAP was attenuated in one. Peroneal compound muscle action potential (CMAP) amplitude (recording from extensor digitorum brevis) was low in five, whereas the tibial CMAP was normal in all patients. Peroneal CMAP amplitude (recording from the tibialis anterior) was normal in three and small in three. Needle electromyography revealed decreased recruitment and fibrillation potentials in L-5-innervated muscles, mostly below the knee. We conclude that intrapartum foot drop occurs mostly in short women and is caused by lumbosacral trunk compression by the fetal head at the pelvic brim. The primary pathology is predominantly demyelination and recovery is complete in up to 5 months.     

Ischaemic lumbosacral plexopathy in acute vascular compromise: case report

Anterior spinal artery syndrome (ASAS) is a well reported cause of spinal cord injury (SCI) following thoracoabdominal aortic surgery. The resultant deficits are often incomplete, typically attributed to the variable nature of the vascular distribution. Our Physical Medicine and Rehabilitation (PM and Rehabilitation) service was consulted about a 36-year-old patient with generalised deconditioning, 3 months after a stab wound to the left ventricle. Physical examination revealed marked lower extremity weakness, hypotonia, hyporeflexia, and a functioning bowel and bladder. Further questioning disclosed lower extremity dysesthesias. Nerve conduction studies showed slowed velocities, prolonged distal latencies and decreased amplitudes of all lower extremity nerves. Electromyography revealed denervation of all proximal and distal lower extremity musculature, with normal paraspinalis. Upper extremity studies were normal. Recently, 3 cases of ischaemic lumbosacral plexopathy, mimicking an incomplete SCI, have been reported. This distinction is particularly difficult in the polytrauma patient with multiple musculoskeletal injuries or prolonged recuperation time, in addition to a vascular insult, as in this patient. The involved anatomical considerations will be discussed. A review of the electrodiagnostic data from 30 patients, with lower extremity weakness following acute ischaemia, revealed a 20% incidence of spinal cord compromise, but no evidence of a plexopathy.     

Intravenous immunoglobulin therapy for idiopathic postoperative lumbosacral plexopathy.

We report a patient who developed an acute lumbosacral plexopathy (LSP) following spinal surgery on lumbos segments. He recovered dramatically following treatment with high-dose intravenous immunoglobulin (IVIg). A 66-year-old man who underwent an L4 to S1 decompressive laminectomy required re-admission after developing contralateral leg pain. Follow-up lumbosacral magnetic resonance imaging showed only mild postoperative changes. Ten days after re-admission, he developed relatively rapid onset ipsilateral inguinal pain and weakness of all his leg muscles with diminished sensation in a lumbosacral plexus distribution. Re-exploration revealed no specific lesion except for adhesions and resulted in no improvement. Following treatment with IVIg (0.4 g/kg daily) for five days, he showed dramatic resolution of motor weakness and pain. There has been no relapse following six months follow-up. Although IVIg treatment does not guarantee a positive response in all types of LSP, it should be considered for severe, rapidly progressive and even for postoperative cases.     

The natural history and management of patients with congenital deficits associated with lumbosacral lipomas

Introduction

Many patients with lumbosacral lipoma are asymptomatic; however, a significant proportion will have neurological deficits present at birth. Implication of these deficits with respect to natural history and management are not well understood.

Methods

A retrospective review of all infants with lumbosacral lipoma seen at BCCH between 1997 and 2013 was carried out. The study population was stratified on the presence of a congenital, non-progressive deficit and subdivided on treatment approach. The subsequent developments of deficits resulting in untethering procedures were recorded.

Results

Of the 44 infants in this study, 24 patients had no neurologic deficit while 20 patients had a fixed, non-progressive deficit evident at birth.Ten of 24 patients without a neurological deficit at birth underwent a prophylactic untethering with 3 eventually requiring repeat untethering after, on average, 62.7 months. Eleven of 14 asymptomatic, monitored patients required untethering for clinical deterioration. Two required a second untethering procedure after 48.7 months.Ten of 20 infants with congenital deficits present at birth underwent prophylactic untethering, and 4 required further surgery after 124 months. Ten patients underwent observation with 8 eventually requiring surgery. Two required repeat untethered after 154 months. The complication rates and operative burden for patients are similar whether prophylactic or delayed surgery is performed.

Conclusion

The presence of congenital neurologic deficit does not affect the likelihood of deterioration in patients managed expectantly; prophylactic detethering of these patients did not prevent delayed neurologic deterioration. Comparing the need for repeat surgery in prophylactically untethered patients with initial untethering of patients operated upon at the time of deterioration, prophylactic untethering may confer a benefit with respect to subsequent symptomatic tethering if complication rates are low. However, in a setting with multidisciplinary follow-up, a period of observation for patients and intervention when patients become symptomatic is an acceptable approach for patients with or without congenital deficits.

     

Failure of immunotherapy to prevent,arrest or reverse diabetic lumbosacral plexopathy

Three patients are described who had severe and progressive diabetic lumbosacral plexopathy despite active immunosuppressive therapy. One patient developed the condition while immunosuppressed for a cardiac transplant and two others progressed while receiving intravenous gamma globulin. The cases raise questions about current unsupported practices of treatment for this condition. Robust clinical trial evidence is required before immunosuppression can be recommended.     

Painful lumbosacral plexopathy with elevated erythrocyte sedimentation rate: a treatable inflammatory syndrome

Six patients had a syndrome of painful lumbosacral plexopathy and elevated erythrocyte sedimentation rate. Sural nerve biopsy in each case showed axonal degeneration and epineurial arterioles surrounded by mononuclear inflammatory cells. Differential fascicular involvement suggested an ischemic cause in three nerves, but no patient had a necrotizing vasculitis. None of the six patients had vasculitis or cancer. Three of the six were diabetic and were initially thought to have diabetic plexopathy, but deterioration continued despite control of the diabetes. These six patients appeared to have an ischemic neuropathy with an immunological basis. Five were treated with immunosuppressant drugs, and in four the plexopathy improved or was arrested. The pathogenesis is unclear, but postmortem findings in one case suggest that the syndrome does not stem from an underlying vasculitis.     

Effect of intravenous immunoglobulin therapy in a case of idiopathic recurrent brachial and lumbosacral plexopathy]

We report a case of a 23-year-old woman with idiopathic brachial and lumbosacral plexopathy who presented a relapsing course of weakness and numbness following acute onset of pain in the right shoulder or pelvic girdle area. On her first admission, the patient recovered gradually with oral prednisolone. But after about 1 year, her symptoms worsened. In the second admission, oral prednisolone had no effect on her symptoms, so intravenous immunoglobulin (IVIG; 0.1 g/kg/day, 5 days) therapy was tried. The patient improved about 2 weeks after IVIG therapy. About 1 year later, her symptoms worsened and was administered again. In the third admission, IVIG therapy of which dose was the same as that in the previous admission had an effect on her symptoms. The dose of IVIG in our case was relatively low, compared with those in cases reported before (0.2-2 g/kg/day). This findings suggest that a relatively low-dose IVIG therapy is effective for idiopathic plexopathy.     

The natural history of dementia

This review summarizes studies on the natural history of dementia with a focus on Alzheimer's disease and vascular dementia. Understanding the course of dementia is important not only for patients, caregivers, and health professionals, but also for health policy‐makers, who have to plan for national resources needed in the management of an increasing number of dementia cases. From the available published data, the life expectancy of elderly people with dementia is shorter than that of non‐demented elderly. Reports on survival after a diagnosis of dementia vary from 3 to 12 years. The wide variation is partly due to the diagnostic criteria used in the studies and the sites where they were conducted (i.e. hospitals, clinics, or homes). There is an apparent difference in survival between Alzheimer's disease patients with onset of illness before 75 years and those after 75 years: the younger patients have a longer life expectancy. However, there are conflicting data on survival (in years) comparing male and female patients and comparing patients of different ethnicities. For vascular dementia, published papers on life expectancy vary between 3 to 5 years. Vascular dementia appears to have a poorer prognosis than Alzheimer's disease. The stages of severity of dementia were compared in a follow‐up of a sample of Alzheimer's disease patients in Singapore, and the mean duration of the mild phase (clinical dementia rating 1) was 5.6 years, the moderate phase (clinical dementia rating 2) was 3.5 years, and the severe phase (clinical dementia rating 3) was 3.2 years. At the various phases of the disease, the demand on health‐care services and economic cost are different.     

Natural history of radiation-induced brachial plexopathy compared with surgically treated patients

Summary Twelve patients who developed radiation-induced brachial plexopathy (RIBP) after receiving radiation therapy for breast carcinoma (7 patients) or Hodgkin's lymphoma (5 patients) were followed for 12 or more years, with a mean follow-up time of 20 years. Tingling and numbness of the fingers as well as weakness of the hand or arm were the most prominent presenting symptoms of RIBP. Whereas pain in most patients evolved only later in their course, it became a predominant feature in only 2. In 8 of the 12 patients, the plexopathy was surgically treated, either by neurolysis only or by neurolysis plus omental grafting in order to stop progression or paresis and/or pain. In 8 patients, including 6 of the operated group, there was slow and steady progression of RIBP over time, with the final outcome being almost complete paralysis of the arm (2 patients) or severe sensorimotor paresis rendering the hand useless (6 patients). In only 4 patients, including 2 of the non-operated group, was there absence of progression and stabilization of the paresis with only slight functional loss of the affected arm in 3 patients and severe palsy in 1. None of the 12 patients had any clear long-lasting improvement of their sensorimotor impairment. It is concluded from this study that RIBP, irrespective of surgery (neurolysis and/or omentum transplant), left two-thirds of the patients with severe or total paresis of the arm. However, the almost complete relief of severe pain (6 of 8 patients), both immediately and in follow-up patients treated with neurolysis and/or omental transplant, indicates that surgical treatment has a beneficial effect on pain relief.     

The natural history of multiple sclerosis

The natural history of chronic diseases is generally poorly understood. Multiple sclerosis os one of the best-studied diseases and we outline here some of the information obtained from 25 000 patient-years of observation. This population-based cohort received almost no treatment and certainly none known to alter the long-term natural course of the illness. There is a possibility that such data will prove useful in new generation clinical trials which are observational in nature.