Screening of hepatitis E in patients presenting for acute neurological disorders.

INTRODUCTION: Hepatitis E virus (HEV) infection has been reported to be associated with neurological disorders. However, the real prevalence of acute hepatitis E in those diseases is still unknown. We determined the prevalence of anti-HEV IgM antibody in a population with acute non-traumatic, non-metabolic, non-vascular neurological injury. METHOD: A registry was created in Grenoble Hospital University from 2014 to 2018 to collect data on patients with acute (<3 months) non-traumatic, non-metabolic, non-vascular neurological injuries. Acute hepatitis E was defined as anti-HEV IgM-positive serum in immunocompetent patient, and as anti-HEV IgM-positive serum or HEV RNA-positive serum in immunocompromised patients. RESULTS: One hundred fifty-nine patients were included. Anti-HEV IgM seroprevalence in our cohort of non-traumatic, non-metabolic, non-vascular neurological injuries was 6.9% (eleven patients, including 4 Parsonage-Turner syndrome (PTS) and 2 Guillain-Barré syndrome (GBS)). Elevated transaminases were observed in only 64% of hepatitis E patients and cholestasis in 64%. CONCLUSION: In this study, 6·9% of patients with acute non-traumatic, non-metabolic, non-vascular neurological injuries had a probable recent HEV infection. HEV serology should be systematically performed in this population, even in patients with normal transaminase level.

Backpack palsy and other brachial plexus neuropathies in the military population.

Brachial plexus neuropathy is often seen in the military population, especially due to pressure (backpack palsy, BPP) or idiopathic (neuralgic amyotrophy, NA). We aimed to gain insight in the disease characteristics of soldiers with brachial plexus neuropathies in the Dutch military population and to compare disease characteristics between patients with BPP and NA. In this retrospective chart review study we aimed to include all patients with brachial plexus neuropathy, who presented in the Joint Military Hospital between 1 January, 2011 and 31 December, 2016. We calculated the incidence of NA and BPP and Chi-square tests or Student t tests were performed for differences in patient characteristics between NA and BPP. We included 127 patients, 63 with BPP, 45 with NA, 10 with traumatic brachial plexus neuropathy, and 9 with other plexopathy. The incidence of brachial plexus neuropathy was 50/100 000 person years overall, 25/100 000 person years for BPP, and 18/100 000 person years for NA. Patients in the BPP group differed from the NA with regard to pain (BPP 41% vs NA 93%, P = .000), atrophy (13% BPP vs 29% NA, P = .049), and sensory symptoms (83% BPP vs 44% NA, P = .000). In the BPP group 90% had incomplete recovery and in the NA group 78%. Our study showed a high incidence of BPP and NA in the military population and suggests recovery is not so benevolent as previously thought. Future research is necessary to improve insight and outcome of military patients with brachial plexus neuropathies.

Neurologic complications of acute hepatitis E virus infection.

OBJECTIVE: To assess the prevalence and clinical features of neurologic involvement in patients with acute hepatitis E virus (HEV) infection in Southern Switzerland. METHODS: Among 1,940 consecutive patients investigated for acute hepatitis E, we identified 141 cases of acute of HEV infection (anti-HEV immunoglobulin M and immunoglobulin G both reactive and/or HEV RNA positive) between June 2014 and September 2017. Neurologic cases were followed up for 6 months. We compared patients with and without neurologic symptoms. RESULTS: Neurologic symptoms occurred in 43 acute HEV cases (30.4%) and consisted of neuralgic amyotrophy (NA, n = 15, 10.6%) and myalgia (n = 28, 19.8%). All NA cases were immunocompetent. Men had higher odds (OR = 5.2, CI 1.12-24.0, p = 0.03) of developing NA after infection with HEV, and in 3 couples simultaneously infected with HEV, only men developed NA. Bilateral involvement of NA was predominant (2:1) and occurred only in men. Seven NA cases were viremic (all genotype 3), but HEV was undetectable in their CSF. In the acute phase of NA, 9 patients were treated with intravenous immunoglobulin and 4 with prednisone, reporting no side effects and improvement in pain and strength. Myalgia occurred both without (n = 16) or with (n = 12) concomitant elevated serum creatinine kinase. Seven cases with myalgia in the shoulder girdle did not have muscle weakness ("forme fruste" of NA). CONCLUSIONS: Neurologic symptoms occurred in one-third of acute HEV infections and consisted of NA and myalgia. NA seems to occur more frequently in men infected by HEV and has a predominant (but not exclusive) bilateral involvement.

Phrenic neuropathy and diaphragm dysfunction in neuralgic amyotrophy.

OBJECTIVE: To describe the clinical phenotype and recovery of diaphragm dysfunction caused by neuralgic amyotrophy in a large cohort of patients, to improve accurate awareness of this entity, and to encourage adoption of a standardized approach for diagnosis and treatment. METHODS: This observational cohort study recruited adult patients with neuralgic amyotrophy and symptoms of idiopathic phrenic neuropathy from the database of the Dutch expert center for neuralgic amyotrophy and the Dutch centers for home mechanical ventilation. Demographic and clinical information on diagnosis, symptoms, and recovery was obtained from chart review. We attempted to contact all patients for a follow-up interview. RESULTS: Phrenic neuropathy occurs in 7.6% of patients with neuralgic amyotrophy. Unilateral diaphragmatic dysfunction and bilateral diaphragmatic dysfunction are frequently symptomatic, causing exertional dyspnea, orthopnea, disturbed sleep, and excessive fatigue. Diagnostic practices varied widely and were often not optimally targeted. The majority of patients experienced at least moderate recovery within 2 years. CONCLUSION: We recommend screening every patient with neuralgic amyotrophy for diaphragm dysfunction by asking about orthopnea and by performing upright and supine vital capacity screening and diaphragm ultrasound in cases of suspected phrenic neuropathy to optimize diagnosis and care.

Hepatitis E virus infection and acute non-traumatic neurological injury: A prospective multicentre study.

BACKGROUND & AIMS: Hepatitis E virus (HEV) has been associated with a number of neurological syndromes, but causality has not yet been established. The aim of this study was to explore the relationship between HEV and neurological illness by prospective HEV testing of patients presenting with acute non-traumatic neurological injury. METHODS: Four hundred and sixty-four consecutive patients presenting to hospital with acute non-traumatic neurological illnesses were tested for HEV by serology and PCR from four centres in the UK, France and the Netherlands. RESULTS: Eleven of 464 patients (2.4%) had evidence of current/recent HEV infection. Seven had HEV RNA identified in serum and four were diagnosed serologically. Neurological cases in which HEV infection was found included neuralgic amyotrophy (n=3, all PCR positive); cerebral ischemia or infarction (n=4); seizure (n=2); encephalitis (n=1); and an acute combined facial and vestibular neuropathy (n=1). None of these cases were clinically jaundiced and median ALT at presentation was 24IU/L (range 8-145). Cases of HEV-associated neuralgic amyotrophy were found in each of the participating countries: all were middle-aged males with bilateral involvement of the brachial plexus. CONCLUSIONS: In this cohort of patients with non-traumatic neurological injury, 2.4% had evidence of HEV infection. Symptoms of hepatitis were mild or absent and no patients were jaundiced. The cases of HEV-associated neuralgic amyotrophy had similarities with other HEV-associated cases described in a large retrospective study. This observation supports a causal relationship between HEV and neuralgic amyotrophy. To further understand the relevance of HEV infection in patients with acute neurological illnesses, case-control studies are warranted. Lay summary: Hepatitis E virus (HEV), as its name suggests, is a hepatotropic virus, i.e. it causes damage to the liver (hepatitis). Our findings show that HEV can also be associated with a range of injury to the nervous system.

[An unrecognized cause of dyspnoea]. / Een miskende oorzaak van dyspneu.

BACKGROUND: Neuralgic amyotrophy is characterised by pain in the neck or shoulder region, followed by neuropathy of both motor and sensory nerves of the brachial plexus. The incidence of this condition is estimated at 1/1000 per year. In a rare variant of the syndrome, involvement of both phrenic nerves can occur, leading to diaphragmatic paralysis and severe orthopnoea. CASE DESCRIPTION: A 67-year-old woman was referred to us with acute orthopnoea. Imaging studies showed bilateral diaphragmatic paralysis, and electromyography (EMG) confirmed neuropathy of both phrenic nerves. The diagnosis was bilateral neuralgic amyotrophy. The patient received nocturnal ventilation support via nasal high flow oxygen therapy. This symptomatic treatment had a positive effect. CONCLUSION: Isolated phrenic nerve neuropathy is a rare variant of neuralgic amyotrophy, leading to orthopnoea. Recovery is slow and frequently incomplete. Supportive treatment with non-invasive ventilation support is necessary to improve the patient's quality of life.

Neuralgic amyotrophy is not the most common neurologic disorder of the shoulder: a 78-month prospective study of 60 neurologic shoulder patients in a specialist shoulder clinic.

BACKGROUND: Neuralgic amyotrophy (NA) was first described in 1948. Traditional literature describes a painful attack with sudden onset, followed by paresis, with varied outcomes. Recent studies have suggested NA is currently underdiagnosed. However, a large number of studies detailing NA originate from a small group of sources. Our study compared the onset, diagnosis, investigation, and treatment of all neurologic shoulder conditions to provide comparable data for these studies. METHODS: Data were collected from 60 patients (81.6% male; median age, 41.5 years) during a 78-month period. Patients with a diagnosis of a neurologic disorder of the shoulder with confirmatory electromyogram (EMG) studies were included. RESULTS: NA was diagnosed in 18 patients before the EMG investigation. Of the clinically diagnosed NA patients, only 5 (27.8%) had EMG findings supportive of NA. A further 5 patients with a clinical diagnosis other than NA were diagnosed with NA after EMG findings. Overall, 10 patients (16.6%) in our study were diagnosed with NA after EMG studies. Only 4 (40.0%) reported a sudden onset attack associated with NA. Supraspinatus and infraspinatus were involved in 9 patients (90.0%), suggesting a predictable distribution of muscle involvement. CONCLUSIONS: These results suggest that NA is overdiagnosed and does not warrant the increased attention suggested by recent articles. The current study also highlights a necessity to perform EMG investigations in all cases of suspected NA because the accuracy of the clinical assessment is poor.

Hepatitis E and neuralgic amyotrophy: Five cases and review of literature.

Hepatitis E virus infection - mainly genotype 3 - is increasingly common in industrialized countries. Infection is usually asymptomatic, but cases of central or peripheral neurological symptoms with hepatitis E have been described. The most frequent is Guillain-Barre but somes cases of neuralgic amyotrophy have been described. In our center, since 2010, we have identified five cases of neuralgic amyotrophy associated with acute hepatitis E in immunocompetent patients. For all these patients, neuralgic amyotrophy was diagnosed with electromyogram and positive IgM for hepatitis E, and detectable HEV RNA in 4 of the cases. Including our patients, we count 26 cases in literature. The mean age of the patients was 44 years old, with a large predominance of males (88%). The disorder is bilateral and asymmetric in 69% of cases. Peripheral nerves other than the brachial plexus were affected in 6 patients (23%). In industrialized countries, any neuralgic amyotrophy, particularly if there is bilateral, asymmetric associated with involvement of nerves outside the brachial plexus, should lead physicians to consider a diagnosis of acute hepatitis E.

Incidence of neuralgic amyotrophy (Parsonage Turner syndrome) in a primary care setting--a prospective cohort study.

OBJECTIVE: Neuralgic amyotrophy is considered a rare peripheral nervous system disorder but in practice seems grossly under recognized, which negatively affects care for these patients. In this study we prospectively counted the one-year incidence rate of classic neuralgic amyotrophy in a primary care setting. METHODS: In a prospective cohort study during the year 2012 we registered all new cases of neck, shoulder or arm complaints from two large primary care centers serving a population of 14,118. Prior to study, general practitioners received a short training on how to diagnose classic neuralgic amyotrophy. Neuralgic amyotrophy was defined according to published criteria irrespective of family history. Only patients with a classic phenotype were counted as definite cases. After inclusion, patients with suspected neuralgic amyotrophy who had not yet seen a neurologist were offered neurologic evaluation for diagnostic confirmation. RESULTS: Of the 492 patients identified with new onset neck, shoulder or arm complaints, 34 were suspected of having neuralgic amyotrophy. After neurologic evaluation the diagnosis was confirmed in 14 patients. This amounts to a one-year incidence rate for classic neuralgic amyotrophy of 1 per 1000. CONCLUSIONS: Our findings suggest that neuralgic amyotrophy is 30-50 times more common than previously thought. Unawareness of the disorder and its clinical presentation seems the most likely explanation for this difference. An incidence rate of 1 per 1000 and the long-term sequelae many patients suffer warrant more vigilance in diagnosing the disorder, to pave the way for timely treatment and prevent complications.

Adjacent segment disease after anterior cervical interbody fusion: a multicenter retrospective study of 288 patients with long-term follow-up.

INTRODUCTION: Cervical discectomy with interbody fusion is a common procedure in spinal surgery. The resultant biomechanical alterations accelerate degeneration of the adjacent segment, but the contribution of natural degeneration to adjacent segment disease is unclear. OBJECTIVE: To assess the long-term rate of surgery to discs adjacent to cervical interbody fusion; and to assess the associated incidence of cervico-brachial neuralgia and radiological degeneration of adjacent discs. MATERIAL AND METHOD: A multicenter retrospective study included anterior cervical discectomy patients at a minimum of 10 years' follow-up. Clinical variables comprised pain, use of analgesics and surgical revision. Functional assessment was performed on the Neck Disability Index (NDI). Radiologic degeneration was assessed on the Goffin score based on cervical spine X-ray. RESULTS: Two hundred and eighty-eight patients were contacted and filled out the clinical questionnaire. Among the patients, 153 underwent radiological reassessment. Mean age was 46 years (range, 16-73 years). Mean follow-up was 14.5 years (12-18 years). The rate of surgical revision on a disc adjacent to the primary level was 5.9%. Frequent attacks of cervico-brachial neuralgia were reported in 20.5% of cases. Radiologic adjacent segment degeneration was found in 81.3% of cases over follow-up. There was a significant correlation between degree of radiologic adjacent segment degeneration and NDI (P=0.02). DISCUSSION: Degeneration adjacent to discectomy/fusion is partly due to aging. The present findings, however, agree with the literature and indicate accelerated degeneration in adjacent segments. These findings should be taken into account in treatment decision-making and suggest a possible interest of more physiological surgery such as arthroplasty. LEVEL OF EVIDENCE: IV - Multicenter retrospective study.

Neuralgic amyotrophy and hepatitis E virus infection.

OBJECTIVE: To determine whether there is an association between an acute preceding hepatitis E virus (HEV) infection and neuralgic amyotrophy (NA), and if so, whether patients with HEV-related NA differ from patients without an associated HEV infection. METHODS: HEV testing was conducted in a retrospective cohort of 28 Cornish patients with NA (2011-2013) and a prospective cohort of 38 consecutive Dutch patients with NA (2004-2007). Acute-phase serum samples were analyzed for the presence of anti-HEV immunoglobulin (Ig) M and IgG and HEV RNA (quantitative real-time PCR). RESULTS: Five cases (10.6%) of acute hepatitis E infection were identified in a total group of 47 patients with NA of whom serum samples were available. In 4 patients, HEV RNA was detected in serum samples taken at presentation. All patients with HEV-associated NA had clinical and electrophysiologic evidence of bilateral brachial plexus involvement. Anti-HEV IgM positivity was not related to age, sex, disease severity, disease course, or outcome. CONCLUSIONS: Acute hepatitis E is found in 10% of patients with NA from the United Kingdom and the Netherlands. Further research is required to investigate the role of HEV in NA in other geographical locations and to determine pathophysiologic mechanisms.

Unusual association of epidemic dropsy with brachial neuritis and palatal palsy.

Epidemic dropsy (ED) results from accidental ingestion of adulterated mustard oil with argemone oil. Chief organs involved in this disease are heart, subcutaneous tissue, eyes and kidneys. Nervous system involvement is very rare. Objective manifestation of neurological involvement is even rarer. The authors report two cases from the same family, who were victims of epidemic dropsy along with their parents. One of them showed objective neurologic involvement in the form of brachial neuritis and another showed palatal palsy.

Brachial neuropraxia in Canadian Atlantic University sport football players: what is the incidence of "stingers"?

OBJECTIVES: The objectives of this study were (1) to determine the incidence of brachial neuropraxia (stingers) among varsity football players during the 2010 season; (2) to determine if associations exist between sustaining a stinger and previous history of stingers, years played, equipment, age, body mass index (BMI), and conditioning; and (3) to provide descriptive statistics regarding stingers and position played, symptoms, activity during injury, mechanism of tackling, and reporting of stingers. DESIGN: Retrospective. SETTING: Canadian Atlantic University Sport football league. PARTICIPANTS: Two hundred forty-four players. ASSESSMENT OF RISK FACTORS: Two written questionnaires. MAIN OUTCOME MEASURES: Number of players experiencing stingers that occurred during the 2010 season. RESULTS: The incidence was 26% (64 of 244). A multivariate analysis revealed that previous history of a stinger (P < 0.0001) and years played (P = 0.0018) were associated with sustaining a stinger. There was no statistically significant effect related to additional equipment, a player's age, BMI, or participation in a strength training program. Linebackers, offensive linemen, and wide receivers had the highest incidence of stingers. The most frequent symptoms reported were tingling, numbness, burning, and weakness. Of all stingers sustained, only 59% (38 of 64) were reported to medical staff. CONCLUSIONS: Stingers are a common injury in Canadian university football and are underreported to medical staff. Education of players at increased risk is needed.

[Cervicobrachialgia - an update under special consideration of the surgical management]. / Zervikobrachialgie - ein Update unter besonderer Berücksichtigung der chirurgischen Therapie.

The classic cervicobrachialgia results acutely from cervical nerve root compression by disc herniation or subacutely by radicular compression after progressive spondylotic changes of the cervical spine. The clinical presentation includes local and radiating pain syndromes that can be accompanied by sensorimotor deficits. Besides the medical history and a targeted clinical examination, supplementary radiographic means should be undertaken to confirm diagnosis. If no urgent surgical indication exists, conservative therapy should be initiated. However, with varying results of conservative and surgical therapy, chronic impairment can occur.

Prevalence of cervicobrachial discomforts in elementary school teachers.

OBJECTIVE: To determine the incidence of cervicobrachial order discomforts in Elementary Public School teachers from 1st to 4th years in the city of Pato Branco - PR. METHODS: Cross-sectional study made with 160 public school teachers from 1st to 4th years in the city of Pato Branco - PR. Data collection was made from a structured questionnaire, and a physical examination with manual palpation and orthopedic tests. RESULTS: The obtained data showed the presence of pain in the trapezius muscle region, on the left side, in 52.5%; and, on the right side, in 50.6%. The analysis also showed that there is a strong relationship between the cervicobrachial problems presented and the labor activity. The final evaluation of this study demonstrates the great need of primary care for these workers, that is, carrying out activities to prevent musculoskeletal diseases developed through work, either to preserve the individuals' physical integrity or the quality of education. CONCLUSIONS: The prevalence of musculoskeletal disorders was high among teachers. There is evidence that the prevalence was connected to job demands.

[Uncommon cervicobrachial neuralgia: about 17 cases]. / Les névralgies cervico-brachiales symptomatiques : Apropos de 17 cas.

BACKGROUND: Cervicobrachial neuralgia (CBN) is frequent in out patient clinic as well in general medicine as in rheumatology. Even though cervical disc degeneration and osteophytosis constitute the most frequent etiology of such a trouble, the practitioner must be aware of much more serious underlying cause. AIM: To investigate the epidemiology, clinical features, aetiologies and paraclinical characteristics of uncommon CBN. METHODS: Retrospective chart review about 17 cases of uncommon CBN among the 84 cases of CBN hospitalized at the rheumatology department of the Charles Nicolle Hospital during a 16-years-period [1990-2005]. RESULTS: There were 11 men and 6 women with a mean age of 63 years [33y-81y]. All patients presented a CBN since in average five months [2 months- 24 months]. Neck stiffness was noted in 60% of cases and a neurological impairment in 47% of cases. X-ray radiographs of cervical spine were normal in five cases. In the remaining cases, they showed lytic images (six cases), disk space narrowing with vertebral erosions (two cases) and vertebral fracture (three cases). Further investigations concluded that the CBN was due to a Pancoast'syndrome in five cases, an infectious spondylitis in three cases, cervical bone metastasis in two cases, a syringomyelia in two cases, a neuroma in one case, a thoracic outlet syndrome in one case and an erosive spondylarthropathy in a patient presenting chronic renal failure managed by hemodialysis. CONCLUSION: In comparison with common CBN, our patients presenting symptomatic CBN were characterised by an inflammatory and refractory pain. The more frequent recourse to modern imaging is justified.

Long-term pain, fatigue, and impairment in neuralgic amyotrophy.

OBJECTIVES: Recently, it has become clear that neuralgic amyotrophy (NA; idiopathic and hereditary brachial plexus neuropathy) has a less optimistic prognosis than usually assumed. To optimize treatment and management of these patients, one needs to know the residual symptoms and impairments they suffer. Therefore, the objective of this study was to describe the prevalence of pain, psychologic symptoms, fatigue, functional status, and quality of life in patients with NA. SETTING: Neurology outpatient department of an academic teaching hospital. PARTICIPANTS: NA patients (N=89) were studied, and clinical details were recorded. Self-report data were on average collected 2 years after the onset of the last NA episode. MAIN OUTCOME MEASURES: Pain was assessed with the McGill Pain Questionnaire, fatigue with the Checklist Individual Strength, and psychologic distress with the Symptom Checklist 90. Functional status and handicap were assessed with the modified Rankin Scale and Medical Outcomes Study 36-Item Short-Form Health Survey. RESULTS: Pain was usually localized in the right shoulder and upper arm, matching the clinical predilection site for paresis in NA. About a quarter to a third of the patients reported significant long-term pain and fatigue, and half to two thirds still experienced impairments in daily life. Over one third of the individual patients suffered from severe fatigue. The group did not fulfill the criteria of chronic fatigue or major psychologic distress. There was no correlation of pain or fatigue with the level of residual paresis on a Medical Research Council scale, but patients with a comorbid condition fared worse than patients without. CONCLUSIONS: A significant number of NA patients suffer from persistent pain and fatigue, leading to impairment. Symptoms were not correlated with psychologic distress. This makes it likely that they are caused by residual shoulder or arm dysfunction but not as part of a chronic pain or fatigue syndrome in these patients.

Hereditary motor and sensory neuropathy with agenesis of the corpus callosum.

Hereditary motor and sensory neuropathy associated with agenesis of the corpus callosum (OMIM 218000) is an autosomal recessive disease of early onset characterized by a delay in developmental milestones, a severe sensory-motor polyneuropathy with areflexia, a variable degree of agenesis of the corpus callosum, amyotrophy, hypotonia, and cognitive impairment. Although this disorder has rarely been reported worldwide, it has a high prevalence in the Saguenay-Lac-St-Jean region of the province of Quebec (Canada) predominantly because of a founder effect. The gene defect responsible for this disorder recently has been identified, and it is a protein-truncating mutation in the SLC12A6 gene, which codes for a cotransporter protein known as KCC3. Herein, we provide the first extensive review of this disorder, covering epidemiological, clinical, and molecular genetic studies.

Groningen Manipulation Study. The effect of manipulation of the structures of the shoulder girdle as additional treatment for symptom relief and for prevention of chronicity or recurrence of shoulder symptoms. Design of a randomized controlled trial within a comprehensive prognostic cohort study.

BACKGROUND: We present the design of the Groningen Manipulation Study. This randomized controlled trial is part of the Dutch Shoulder Disability Study, a comprehensive prognostic cohort study on shoulder disorders, with randomized controlled interventions in subcohorts. OBJECTIVE: To evaluate the effectiveness of manipulative treatment of the structures of the shoulder girdle, in addition to standard treatment by the general practitioner for relief of shoulder symptoms and prevention of persistent or recurrent shoulder symptoms. METHODS: A total of 250 patients with shoulder symptoms and a functional limitation of the shoulder girdle will be included from 30 general practices in Groningen, The Netherlands. All participating patients receive standard treatment by the general practitioner and will be randomly allocated to additional manipulative treatment. Evaluation measurements take place 6, 12, 26, and 52 weeks after randomization. CONCLUSION: The short-term primary outcome measure is the proportion of patients with relief of shoulder complaints and the long-term primary outcome is the proportion of patients without persistent or recurrent shoulder symptoms. Dependent and independent variables include a structured medical history, a physical examination of the shoulder and shoulder girdle, and a measure of the mobility of the cervico-thoracic spine with a 6-degree-of-freedom electromagnetic tracking device.