Illness Weakness, Polyneuropathy and Myopathy: Diagnosis, treatment, and long-term outcomes.

BACKGROUND: Severe weakness associated with critical illness (CIW) is common. This narrative review summarizes the latest scientific insights and proposes a guide for clinicians to optimize the diagnosis and management of the CIW during the various stages of the disease from the ICU to the community stage. MAIN BODY: CIW arises as diffuse, symmetrical weakness after ICU admission, which is an important differentiating factor from other diseases causing non-symmetrical muscle weakness or paralysis. In patients with adequate cognitive function, CIW can be easily diagnosed at the bedside using manual muscle testing, which should be routinely conducted until ICU discharge. In patients with delirium or coma or those with prolonged, severe weakness, specific neurophysiological investigations and, in selected cases, muscle biopsy are recommended. With these exams, CIW can be differentiated into critical illness polyneuropathy or myopathy, which often coexist. On the general ward, CIW is seen in patients with prolonged previous ICU treatment, or in those developing a new sepsis. Respiratory muscle weakness can cause neuromuscular respiratory failure, which needs prompt recognition and rapid treatment to avoid life-threatening situations. Active rehabilitation should be reassessed and tailored to the new patient's condition to reduce the risk of disease progression. CIW is associated with long-term physical, cognitive and mental impairments, which emphasizes the need for a multidisciplinary model of care. Follow-up clinics for patients surviving critical illness may serve this purpose by providing direct clinical support to patients, managing referrals to other specialists and general practitioners, and serving as a platform for research to describe the natural history of post-intensive care syndrome and to identify new therapeutic interventions. This surveillance should include an assessment of the activities of daily living, mood, and functional mobility. Finally, nutritional status should be longitudinally assessed in all ICU survivors and incorporated into a patient-centered nutritional approach guided by a dietician. CONCLUSIONS: Early ICU mobilization combined with the best evidence-based ICU practices can effectively reduce short-term weakness. Multi-professional collaborations are needed to guarantee a multi-dimensional evaluation and unitary community care programs for survivors of critical illnesses.

Orthodontic treatment of a patient with unilateral orofacial muscle dysfunction: The efficacy of myofunctional therapy on the treatment outcome.

The orofacial muscle is an important factor in the harmony of the occlusion, and its dysfunction significantly influences a patient's occlusion after craniofacial growth and development. In this case report, we describe the successful orthodontic treatment of a patient with unilateral orofacial muscle dysfunction. A boy, 10 years 0 months of age, with a chief complaint of anterior open bite, was diagnosed with a Class III malocclusion with facial musculoskeletal asymmetry. His maxillomandibular relationships were unstable, and he was unable to lift the right corner of his mouth upon smiling because of weak right orofacial muscles. A satisfactory occlusion and a balanced smile were achieved after orthodontic treatment combined with orofacial myofunctional therapy, including muscle exercises. An acceptable occlusion and facial proportion were maintained after a 2-year retention period. These results suggest that orthodontic treatment with orofacial myofunctional therapy is an effective option for a patient with orofacial muscle dysfunction.

ICU-acquired weakness: what is preventing its rehabilitation in critically ill patients?

Intensive care unit-acquired weakness (ICUAW) has been recognized as an important and persistent complication in survivors of critical illness. The absence of a consistent nomenclature and diagnostic criteria for ICUAW has made research in this area challenging. Although many risk factors have been identified, the data supporting their direct association have been controversial. Presently, there is a growing body of literature supporting the utility and benefit of early mobility in reducing the morbidity from ICUAW, but few centers have adopted this into their ICU procedures. Ultimately, the implementation of such a strategy would require a shift in the knowledge and culture within the ICU, and may be facilitated by novel technology and patient care strategies. The purpose of this article is to briefly review the diagnosis, risk factors, and management of ICUAW, and to discuss some of the barriers and novel treatments to improve outcomes for our ICU survivors.

The chronically critically ill patient: pediatric considerations.

Whether defined as chronically critically ill, long-term mechanical ventilator dependent (or otherwise chronically medically supported), or medically fragile, a population of infants and children with chronic illness clearly exists. Infants and children with chronic healthcare needs are at an increased risk for physical, developmental, behavioral, and/or emotional conditions and generally require healthcare services of a type or amount beyond that of a general pediatric or adult population. This review will focus on the specific management and psychosocial needs associated with the healthcare of this subgroup of infants and children with chronic illness. Attention will be paid to defining the population, describing trends over time, reviewing their special needs, and discussing outcomes. Increased focus and an increasing quantity of resources for this subgroup of infants and children are needed, as the number of such pediatric patients continues to grow.

[To evaluate the therapeutic efficacy of hemoperfusion in treating intermediate syndrome following acute organophosphate poisoning].

OBJECTIVE: To evaluate the therapeutic efficacy of hemoperfusion in the treatment of intermediate myasthenia syndrome (IMS) following acute organophosphate poisoning (AOPP). METHODS: Eighty cases of IMS following AOPP, who were admitted to the Emergency Department of our hospital from 2006 to 2011 and had complete clinical records, were divided into HP treatment group (n = 36) and non-HP (NHP) treatment group (n = 44). The therapeutic efficacy of HP was evaluated by comparing the clinical data of the two groups. RESULTS: The HP treatment group showed significantly increased serum cholinesterase activity at 24h and 72 h after admission (P < 0.05), while the NHP treatment group showed significantly increased serum cholinesterase activity at 72 h after admission (P < 0.05). The serum cholinesterase activity in the HP treatment group was significantly higher than that in the NHP treatment group at 24 h after admission (P < 0.05). Compared with the NHP treatment group, the HP treatment group had significantly decreased total atropine dose, time of ventilatory assistance, length of ICU stay, recovery time from coma, incidence of pulmonary infection, and mortality due to respiratory failure (P < 0.05). There were no significant differences in the incidence of upper gastrointestinal hemorrhage and total mortality between the two groups (P > 0.05). CONCLUSION: Hemoperfusion is an effective therapy for improving clinical symptoms, shorten the course of disease, reducing complications, and decreasing the mortality due to respiratory failure in the patients with IMS following AOPP.

Neuromuscular Electrical Stimulation Improves Muscle Strength, Biomechanics of Movement, and Functional Mobility in Children With Chronic Neurological Disorders: A Systematic Review and Meta-Analysis.

OBJECTIVE: Chronic neurological disorders (CNDs) generally produce deleterious effects on the musculoskeletal system and can affect physical activity and increase sedentary behavior in children, hindering the execution of training programs and the attainment of a correct dose of exercise. The purpose of this systematic review was to analyze the effect of neuromuscular electrical stimulation (NMES) on skeletal muscle and then on biomechanics of movement, functional mobility, strength, spasticity, muscle architecture, and body composition of children and adolescents with CNDs and chronic diseases. METHODS: The search was conducted in April 2020 in PubMed, MEDLINE, Scopus, the Cochrane Library, and Web of Science, without publication period restriction. Publications investigating the effect of NMES on children and adolescents with CNDs and other chronic diseases were independently selected by 2 researchers. One author independently extracted data from the studies selected, and a second author cross-checked. RESULTS: Eighteen studies with 595 participants aged between 3 and 14 years were included. Quality assessment showed that 50% of the studies presented a low risk of bias. The pooled effect of NMES on gross motor functional measure, calculated as a standardized mean difference using a random effects model, was 0.41 (95% CI = 0.19-0.64). CONCLUSION: The use of NMES programs for children diagnosed with cerebral palsy, spinal muscular atrophy, and obstetric injury of the brachial plexus was effective in improving muscle strength, biomechanics of movement, and functional mobility. IMPACT: NMES can be a useful tool to prevent the reduction of mobility that results from CNDs.

Rehabilitation in practice: management of lower motor neuron weakness.

This series of articles for rehabilitation in practice aims to cover a knowledge element of the rehabilitation medicine curriculum. Nevertheless they are intended to be of interest to a multidisciplinary audience. The competency addressed in this article is 'The trainee consistently demonstrates a knowledge of the pathophysiology of various specific impairments including lower motor neuron weakness' and 'management approaches for specific impairments including lower motor neuron weakness'.This article explores weakness as a lower motor symptom. Weakness as a primary impairment of neuromuscular diseases is addressed, with recognition of the phenomenon of disuse atrophy, and how weakness impacts on the functional abilities of people with myopathy and neuropathy. Interventions to reduce weakness or address the functional consequences of weakness are evaluated with consideration of safety and clinical application. LEARNING OUTCOMES: This paper will allow readers to: (1) appraise the contribution of research in rehabilitation of lower motor neuron weakness to clinical decision making and (2) engage with the issues that arise when researching rehabilitation interventions for lower motor neuron weakness. AIM OF ARTICLE: Impairments associated with neuromuscular conditions can lead to significant functional difficulties that can impact on a person's daily participation. This article focuses on the primary impairment of weakness and explores the research evidence for rehabilitation interventions that directly influence weakness or address the impact of weakness on function.

Rehabilitation following lumbosacral percutaneous nucleoplasty: a case report.

STUDY DESIGN: Case report. BACKGROUND: Lumbar spine nucleoplasty is a new surgical option for patients with disc pathology. There are no reports in the literature describing the role of physical therapy in postoperative lumbar nucleoplasty management. The purpose of this case is to describe the postoperative physical therapy management of a patient who underwent this procedure. CASE DESCRIPTION: A 50-year-old male, 7 weeks following a L5/S1 lumbar nucleus replacement, completed 6 weeks of rehabilitation. The focus of the treatment was controlled reloading of the spine through a spinal stabilization progression in weight-bearing and non-weight-bearing activities. In addition, education, spinal manual therapy techniques, and a home exercise program were also incorporated. OUTCOMES: The patient's Oswestry Disability Index decreased from 56% to 4% over 6 weeks of treatment. When contacted at 6, 12, 18, and 24 months posttherapy, his Oswestry Disability Index was 2%, 2%, 0%, and 0%, respectively, and he had returned to all previous activities without recurrence of symptoms. DISCUSSION: This case report outlines the clinical decision-making process during the postoperative management of an individual who had undergone a single-level lumbar nucleoplasty. A postoperative regimen of education, segmental spinal stabilization, and a home exercise program might have contributed to the observed improvement in pain and disability levels in this patient. The role of these postoperative interventions warrants further research. LEVEL OF EVIDENCE: Therapy, level 4.

Long-term efficacy of intravenously administered immunoglobulin in a case of polymyositis with limited application of steroid therapy.

A 72-year-old man who had been diagnosed with polymyositis (PM) was admitted to our hospital for pneumonia with exacerbation of muscle weakness, elevation of muscle enzymes, and positive magnetic resonance imaging findings. The patient had been refractory to cyclosporine A or azathioprine and hoped to avoid administration of high-dose steroids; IV immunoglobulin (IVIG) was therefore administered after improvement of the pneumonia. Two weeks after the IVIG therapy, muscle test scores, activities of daily living, and muscle enzymes were drastically improved. Twenty months after IVIG, no relapse of PM was observed.

Fatigue during breast cancer radiotherapy: an initial randomized study of cognitive-behavioral therapy plus hypnosis.

OBJECTIVE: The study purpose was to test the effectiveness of a psychological intervention combining cognitive-behavioral therapy and hypnosis (CBTH) to treat radiotherapy-related fatigue. DESIGN: Women (n = 42) scheduled for breast cancer radiotherapy were randomly assigned to receive standard medical care (SMC) (n = 20) or a CBTH intervention (n = 22) in addition to SMC. Participants assigned to receive CBTH met individually with a clinical psychologist. CBTH participants received training in hypnosis and CBT. Participants assigned to the SMC control condition did not meet with a study psychologist. MAIN OUTCOME MEASURES: Fatigue was measured on a weekly basis by using the fatigue subscale of the Functional Assessment of Chronic Illness Therapy (FACIT) and daily using visual analogue scales. RESULTS: Multilevel modeling indicated that for weekly FACIT fatigue data, there was a significant effect of the CBTH intervention on the rate of change in fatigue (p < .05), such that on average, CBTH participants' fatigue did not increase over the course of treatment, whereas control group participants' fatigue increased linearly. Daily data corroborated the analyses of weekly data. CONCLUSION: The results suggest that CBTH is an effective means for controlling and potentially preventing fatigue in breast cancer radiotherapy patients.

A study of the effects of 8-week acupuncture treatment on patients with Parkinson's disease.

BACKGROUND: Parkinson's disease (PD) is a degenerative brain disorder, resulting in decreased neural responses in the supplementary motor area, putamen, and thalamus. Previous research showed that acupuncture was able to improve the motor dysfunction. The primary aim of this study is to assess the efficacy of longer acupuncture treatment for preventing brain degeneration in patients with PD. METHODS: Ten outpatients with PD were recruited from Kyung Hee Medical Hospital. Behavioral and neural responses were examined before and after 8 weeks of acupuncture treatment. A semi-individualized treatment approach was used; patients were treated for 15 minutes with 120-Hz electro-acupuncture at the right GB34 and Taechung (LR3), followed by manual acupuncture based on the individual symptoms of the patient. RESULTS: Immediately after 8 weeks of acupuncture treatment, the Unified Parkinson's Disease Rating Scale (UPDRS) sub-scores and the depression scores for the patients had statistically decreased compared to the scores before acupuncture treatment; moreover, 8 weeks later, these scores remained stable. Compared to the neural responses before the acupuncture stimulation, those after the acupuncture treatment were significantly higher in the thalamus, cingulate gyrus, anterior cingulate, lingual gyrus, parahippocampal gyrus, lateral globus pallidus, mammillary body, middle temporal gyrus, cuneus, and fusiform gyrus. Finally, a positive correlation was found between the UPDRS and the mean magnetic resonance signal change for the thalamus. CONCLUSION: This study found beneficial clinical effects of 8-week acupuncture treatment in the brains of patients with PD.

[Dropped head syndrome as first manifestation of primary hyperparathyroid myopathy].

75 years old woman presented with 6-month history of progressive dropped head syndrome. Neurological examination revealed moderate weakness of flexor and extensor of neck and mild weakness of proximal appendicular muscles with normal deep tendon reflexes. The needle electromyography showed short duration and low amplitude motor unit potential. No fibrillation potentials or positive sharp waves were seen. Biopsy of deltoid muscle was normal. Laboratory studies showed elevated levels of serum calcium (11.8 mg/dl, upper limit of normal 10.1) and intact parathyroid hormone (104 pg/ml, upper limit of normal 65), and decreased level of serum phosphorus (2.3 mg/dl, lower limit of normal 2.7). Ultrasonography and enhanced computed tomography revealed a parathyroid tumor. The tumor was removed surgically. Pathological examination proved tumor to be parathyroid adenoma. Dropped head and weakness of muscles were dramatically improved within a week after the operation. Although hyperparathyroidism is a rare cause of dropped head syndrome, neurologists must recognize hyperparathyroidism as a treatable cause of dropped head syndrome.

Intensive Care Unit-Acquired Weakness: Implications for Case Management.

PURPOSE: The purpose of this case study is to provide a specific example of the disease trajectory for one patient's experience with intensive care unit-acquired weakness (ICUAW). This case study provides those in case management with an overview of some of the common signs and symptoms of ICUAW, as well as the possible prognosis and recovery from ICUAW. PRIMARY PRACTICE SETTING: The events in this case study take place in the acute care setting including the intensive care unit of a mid-sized health center, a general medical-surgical (med-surg) unit, and a long-term acute care facility. CONCLUSIONS: ICUAW affects the clinical, functional, and financial outcomes of patients. If the patient survives, their quality of life and the quality of life of their family members could be severely impacted. Case management practice has a significant role in coordinating care for those diagnosed with ICUAW. Case managers can use knowledge about ICUAW to improve the patient's transition throughout the hospital stay, improve discharge recommendations, and improve the patient's short-term and long-term outcomes. This may reduce unnecessary utilization of health care resources.

Severe acute inflammatory demyelinating polyradiculoneuropathy with persistent weakness associated with tumor-like nerve root enlargement.

We report a 23-year-old woman with rapid onset of proximal and distal limb weakness and areflexia, associated with tumor-like spinal nerve root enlargement and markedly elevated cerebrospinal fluid protein. Our patient developed the inability to walk within days, without preceding illness. Within two weeks, she had near-complete bilateral wrist and foot drop. Her cranial nerves and respiratory function remained intact. She received intravenous immunoglobulin early on for suspected Guillain-barre syndrome but remained wheelchair-bound until 6 Plasma exchange sessions were completed. After that, she continued to improve with intravenous immunoglobulin dosed every 3-4 weeks. Prominent demyelinating features were found on NCS, with cerebrospinal fluid protein of 415 mg/dL. Comprehensive infectious work-up was negative. Magnetic resonance imaging of lumbosacral and cervical spine showed tumor-like masses mistaken for neurofibromatosis (axial diameter, 7.5-10 mm). Repeated magnetic resonance imaging 6 months later showed persistent nerve root enlargement, despite the patient's improved functional status.

Facial Weakness, Otalgia, and Hemifacial Spasm: A Novel Neurological Syndrome in a Case-Series of 3 Patients With Rheumatic Disease.

Bell palsy occurs in different rheumatic diseases, causes hemifacial weakness, and targets the motor branch of the 7th cranial nerve. Severe, persistent, and refractory otalgia having features of neuropathic pain (ie, burning and allodynic) does not characteristically occur with Bell palsy. Whereas aberrant regeneration of the 7th cranial nerve occurring after a Bell palsy may lead to a variety of clinical findings, hemifacial spasm only rarely occurs. We identified in 3 rheumatic disease patients (2 with Sjögren syndrome, 1 with rheumatoid arthritis) a previously unreported neurological syndrome of facial weakness, otalgia with neuropathic pain features, and hemifacial spasm. We characterized symptoms, examination findings, and response to therapy. All 3 patients experienced vertigo, as well as severe otalgia which persisted after mild facial weakness had completely resolved within 1 to 4 weeks. The allodynic nature of otalgia was striking. Two patients were rendered homebound, as even the barest graze of outdoor breezes caused intolerable ear pain. Patients developed hemifacial spasm either at the time of or within 3 months of facial weakness. Two patients had a polyphasic course, with recurrent episodes of facial weakness and increased otalgia. In all cases, otalgia and hemifacial spasm were unresponsive to neuropathic pain regimens, but responded in 1 case to intravenous immunoglobulin therapy. No patients had vesicles or varicella zoster virus in spinal-fluid studies. We have defined a novel neurological syndrome in 3 rheumatic disease patients, characterized by facial weakness, otalgia, and hemifacial spasm. As described in infectious disorders, the combination of otalgia, facial weakness, and 8th cranial nerve deficits suggests damage to the geniculate ganglia (ie, the sensory ganglia of the 7th cranial nerve), with contiguous involvement of other cranial nerves causing facial weakness and vertigo. However, the relapsing nature and association with hemifacial spasm constitute a unique part of this neurological syndrome.

Catamenial mononeuropathy and radiculopathy: a treatable neuropathic disorder.

OBJECT: The aim of this study was to investigate the indications and treatment options in patients with lower-extremity neuropathies and radiculopathies caused by endometriosis. METHODS: The authors identified five patients whose symptoms included catamenial pain, weakness, and sensory loss involving the sciatic and femoral nerves and multiple lumbosacral nerve roots. Radiographic studies supported the diagnosis of catamenial neuropathy or radiculopathy, but definitive diagnosis depended on surgical and pathological examination. Treatment of symptoms, including physical therapy and a course of antiinflammatory or analgesic medication, was not helpful. Patients responded favorably to hormonal therapy. Laparoscopy or open exploration for extrapelvic lesions was performed for diagnosis or for treatment when hormone therapy failed. Pain and sensory symptoms responded well to therapy. Weakness improved, but never recovered completely. CONCLUSIONS: Catamenial neuropathy or radiculopathy should be considered when evaluating reproductive-age women with recurring focal neuropathic leg pain, weakness, and sensory loss.

Severe respiratory muscle weakness related to long-term colchicine therapy.

We report a case of colchicine-induced myopathy, in which the initial presenting and predominant clinical feature was respiratory muscle dysfunction; there was also chronic renal failure and electromyographically-measured myopathy. Discontinuation of colchicine led to marked improvement. Colchicine discontinuation was the only therapy performed (other medications were unchanged), and within 3 weeks the patient had regained motor function and resumed daily activities. Myopathy from primary biliary cirrhosis was ruled out. In contrast to acute colchicine intoxication, chronic colchicine toxicity is related to prolonged use rather than colchicine serum level, so colchicine serum level was not measured and did not affect the decision to discontinue colchicine. Although the diagnosis was not confirmed by muscle biopsy, we believe the typical presentation and the rapid improvement after withdrawing colchicine confirm the diagnosis. We conclude that long-term colchicine therapy, especially in the setting of chronic renal failure, can produce symptomatic respiratory muscle weakness.

New-onset sensory loss or alteration.

New-onset sensory loss often poses a problem for emergency physicians because of the vast array of potential causes for this condition and the imprecision patients display in explaining their condition. This article reviews potential causes of particular interest to the emergency physician and illustrates approaches to sensory alteration complaint with case studies.

Intermittent cervical traction and thoracic manipulation for management of mild cervical compressive myelopathy attributed to cervical herniated disc: a case series.

STUDY DESIGN: Case series. OBJECTIVE: To describe the management of 7 patients with grade 1 cervical compressive myelopathy attributed to herniated disc using intermittent cervical traction and manipulation of the thoracic spine. BACKGROUND: Intermittent cervical traction has been indicated for the treatment of patients with herniated disc and has been suggested to be helpful for patients with cervical compressive myelopathy. Manipulation of the thoracic spine has been utilized to safely improve active range of motion and decrease pain in patients with neck pain. METHODS AND MEASURES: Seven women with neck pain, 35 to 45 years of age, were identified as having signs and symptoms consistent with grade 1 cervical compressive myelopathy. Symptom duration ranged from less than 1 week to 52 weeks. All patients were treated with intermittent cervical traction and thoracic manipulation for a median of 9 sessions (range, 2-12 sessions) over a median of 56 days (range, 14-146 days). Numeric Pain Rating Scale and Functional Rating Index scores served as the primary outcome measures. RESULTS: The median decrease in pain scores was 5 (range, 2-8) from a baseline of 6 (range, 4-8), and median improvement in Functional Rating Index scores was 26% (range, 10%-50%) from a baseline of 44% (range, 35%-71%). Dizziness was eliminated in 3 out of 4 patients and chronic headache symptoms were improved in 3 out of 3 patients. There were no adverse events or outcomes. CONCLUSIONS: Intermittent cervical traction and manipulation of the thoracic spine seem useful for the reduction of pain scores and level of disability in patients with mild cervical compressive myelopathy attributed to herniated disc. A thorough neurological screening exam is recommended prior to mechanical treatment of the cervical spine.