Acute-onset polyradiculoneuropathy after SARS-CoV2 vaccine in the West and North Midlands, United Kingdom.

INTRODUCTION/AIMS: We aimed to determine whether specific severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) vaccines may be associated with acute-onset polyradiculoneuropathy and if they may result in particular clinical presentations. METHODS: We retrospectively reviewed records of all persons presenting with acute-onset polyradiculoneuropathy from January 1, 2021, to June 30, 2021, admitted to two Neuroscience centers, of the West and North Midlands, United Kingdom. We compared subjects with previous SARS-CoV2 vaccine exposure with a local cohort of persons with acute-onset polyradiculoneuropathy admitted between 2005 and 2019 and compared admission numbers for the studied time frame with that of the previous 3 years. RESULTS: Of 24 persons with acute-onset polyradiculoneuropathy, 16 (66.7%) presented within 4 weeks after first SARS-CoV2 vaccine. Fourteen had received the AstraZeneca vaccine and one each, the Pfizer and Moderna vaccines. The final diagnosis was Guillain-Barré syndrome (GBS) in 12 and acute-onset chronic inflammatory demyelinating polyneuropathy in 4. Among AstraZeneca vaccine recipients, facial weakness in nine persons (64.3%), bulbar weakness in seven (50%), and the bifacial weakness and distal paresthesias GBS variant in three (21.4%), were more common than in historical controls (P = .01; P = .004, and P = .002, respectively). A 2.6-fold (95% confidence interval: 1.98-3.51) increase in admissions for acute-onset polyradiculoneuropathy was noted during the studied time frame, compared to the same period in the previous 3 years. DISCUSSION: Despite a low risk, smaller than that of SARS-CoV2 infection and its complications, exposure to the first dose of AstraZeneca SARS-CoV2 vaccine may be a risk factor for acute-onset polyradiculoneuropathy, characterized by more common cranial nerve involvement.

Tremor in inflammatory neuropathies.

BACKGROUND: Tremor is known to occur in patients with neuropathies although its reported prevalence varies widely. Tremor has been shown to cause disability in children with Charcot-Marie-Tooth disease but no data exit about the disability caused by tremor in inflammatory neuropathies. Little is known about the response of neuropathic tremor to treatment and why it selectively occurs in some people and not others. METHODS: This case control study investigates the presence and severity of tremor in 43 consecutively recruited patients with inflammatory neuropathies at the National Hospital for Neurology and Neurosurgery, London. Clinical assessment, including Fahn-Tolosa-Marin Scale for tremor, sensory scores, power scores and Overall Neuropathy Limitations Scale, were recorded. Results of nerve conduction studies were retrieved and assessed. Nine patients' tremors were recorded with accelerometry. RESULTS: Tremor was most common in IgM paraproteinaemic neuropathies, as previously reported, but also occurred in 58% of those with chronic inflammatory demyelinating polyradiculoneuropathy and 56% of those with multifocal motor neuropathy with conduction block. We describe, for the first time, tremor in the majority of patients with multifocal motor neuropathy with conduction block. Tremor in all of these patients seems generally refractory to treatment except in a small number of cases where tremor improves with treatment of the underlying neuropathy. We provide evidence that tremor may add to disability in patients with inflammatory neuropathy. Mean tremor frequency was 6 Hz and did not vary with weight loading. We demonstrate for the first time that although tremor severity correlates with F wave latency, it is not sufficient to distinguish those with, from those without, tremor. CONCLUSION: Tremor in inflammatory neuropathies is common, adds to disability and yet does not often respond to treatment of the underlying neuropathy. When present, tremor severity is associated with F wave latency.

Neurologic disorders and hepatitis E, France, 2010.

We report meningitis with diffuse neuralgic pain or polyradiculoneuropathy associated with PCR-documented acute hepatitis E in 2 adults. These observations suggest that diagnostic testing for hepatitis E virus should be conducted for patients who have neurologic symptoms and liver cytolysis.

Auto-immune polyradiculoneuropathy and a novel IgG biomarker in workers exposed to aerosolized porcine brain.

Twenty-four patients, all of whom were exposed to aerosolized porcine brain tissue through work-place environment (abattoir), developed a syndrome of immune-mediated polyradiculoneuropathy; three also had central nervous system manifestations (transverse myelitis, meningoencephalitis, and aseptic meningitis). Patients had characteristic electrophysiological findings of very distal and proximal conduction slowing (prolonged distal and F-wave latencies, regions where the blood-nerve barrier is the most permeable) and all patients' serum contained a novel IgG immunofluorescence pattern. Nerve pathology, when available, showed mild changes of segmental demyelination, axonal degeneration, and inflammatory changes. Patients had meaningful improvement of symptoms and electrophysiologic findings with immune therapy and with removal of exposure to aerosolized brain tissue. We postulate that this outbreak is an auto-immune polyradiculoneuropathy triggered by occupational exposure to multiple aerosolized porcine neural tissue antigens that result in neural damage where the blood-nerve barrier is the least robust.

Lumbosacral radiculoplexus neuropathy: Incidence and the association with diabetes mellitus.

OBJECTIVE: To determine the previously unknown incidence of lumbosacral radiculoplexus neuropathy (LRPN) and its association with diabetes mellitus (DM). METHODS: LRPN defined by clinical and electrophysiologic criteria was identified among Olmsted County, Minnesota, residents during a 16-year period (2000-2015) using the unique facilities of the Rochester Epidemiology Project. DM was ascertained using American Diabetes Association criteria. RESULTS: Of 1,892 medical records reviewed, 59 patients (33 men, 26 women) were identified as having LRPN. The median age was 70 years (range 24-88 years) and the median time of onset of symptoms to diagnosis was 2 months (range 1-72 months). DM was more frequent in patients with LRPN than in controls (39/59 vs 35/177, p < 0.001) but not in those with pre-DM (10/20 vs 55/142, p = 0.336). LRPN recurred in 3 patients with DM resulting in 62 LRPN episodes during the study period. The overall incidence of LRPN was 4.16/100,000/y (95% confidence interval [CI] 3.13-5.18). The incidences of LRPN among DM and non-DM groups were 2.79/100,000/y (95% CI 1.94-3.64) and 1.27/100,000/y (95% CI 0.71-1.83), respectively. The odds of LRPN among patients with DM and pre-DM was 7.91 (95% CI 4.11-15.21) and 1.006 (95% CI 1.004-1.012), respectively. CONCLUSIONS: LRPN incidence in Olmsted County of 4.16/100,000/y makes LRPN a common inflammatory neuropathy and is higher than that of other immune-mediated neuropathies (acute or chronic inflammatory demyelinating polyradiculoneuropathy, brachial plexus neuropathy) assessed within the same population. DM is a major risk factor for LRPN and thus justifies the continued classification of LRPN into diabetic and nondiabetic forms.

Amphiphysin-IgG autoimmune neuropathy: A recognizable clinicopathologic syndrome.

OBJECTIVE: To define the clinicopathologic features of amphiphysin-immunoglobulin G (IgG)-mediated neuropathy. METHODS: Patients examined at our institution from January 1, 1995, to September 30, 2018, with amphiphysin-IgG by indirect immunofluorescence and Western blot, were reviewed. Their phenotypes were compared to cases of coexisting collapsin response-mediator protein-5 (CRMP5)-IgG or anti-neuronal nuclear antibody type 1 (ANNA1-IgG) and CRMP5-IgG autoimmunity. Improvement in modified Rankin Scale (mRS) (≥1) on follow-up was considered a favorable outcome. Amphiphysin RNA expression was assessed in healthy nerves. RESULTS: Fifty-three amphiphysin-IgG-positive cases were identified. Of 33 (60%) patients with neuropathy, 21 had amphiphysin-IgG alone, and 12 had coexisting autoantibodies (ANNA1-IgG, n = 8; CRMP5-IgG, n = 2; ANNA1-IgG and CRMP5-IgG, n = 2). The neuropathies in isolated amphiphysin-IgG autoimmunity included polyradiculoneuropathy (62%), diffuse sensory neuronopathy (35%), and facial neuropathy with gastroparesis (3%). Among these, pain (80%), breast cancer (63%), and CNS (57%) involvements commonly coexisted, and neuropathy frequently prompted breast cancer diagnosis (76%). Stiff-person spectrum disorder was the most common CNS accompaniment (45%). Nerve biopsies showed axonal loss (n = 6/6), subperineurial edema (n = 4/6), and CD4 predominant inflammation (n = 2/6). Median mRS score at last follow-up was 3.5; 58% of patients were immunotherapy-responsive. Patients with amphiphysin-IgG alone had more favorable immunotherapy response than patients with CRMP5-IgG polyneuropathy (n = 45) (44% vs 16%, p = 0.028, odds ratio 4.2, 95% confidence interval 1.1 to 15.5). Only 1/9 (11%) patients with amphiphysin-IgG with coexisting CRMP5-IgG or ANNA1-IgG had immunotherapy response. RNA amphiphysin expression occurred at low levels in nerve. CONCLUSION: Amphiphysin-IgG autoimmune neuropathy has a recognizable phenotype, is frequently immune responsive, and can prompt early diagnosis of breast cancer.

An exploratory study into factors influencing development of acute canine polyradiculoneuritis in the UK.

OBJECTIVES: To investigate whether the development of acute canine polyradiculoneuritis is associated with various putative risk factors. MATERIALS AND METHODS: Retrospective case-control study with conditional logistic regression analysis from a referral hospital population in the UK where controls were matched for year of presentation. RESULTS: Forty-three cases were identified with acute canine polyradiculoneuritis and 86 controls were selected. Jack Russell terriers and West Highland white terriers were found to have a significantly greater odds of developing acute canine polyradiculoneuritis compared to a mixed baseline group of dogs. The odds of developing acute canine polyradiculoneuritis were greater in the autumn and winter compared to spring. Vaccination, rural/urban habitation, sex, neuter status and age were not associated with development of acute canine polyradiculoneuritis in our population of dogs. CLINICAL SIGNIFICANCE: Breed and season were associated with development of acute canine polyradiculoneuritis. However, this is a small sample and so this observation needs confirmation in further studies and other factors may also be involved. Nevertheless, these findings may be important in further understanding the aetiopathogenesis of this condition.

Occurrence of nervous system involvement in SIRS.

Systemic inflammatory response syndrome (SIRS) is a medical condition in which the all-organ microcirculation is affected including nervous system. We describe neurological findings in 64 patients with SIRS at Hospital das Clínicas of Sao Paulo University School of Medicine; 45.3% were male and 54.7% female; their age ranged from 16 to 95 years old. SIRS was caused by infection in 68.8% of patients, trauma in 10.9%, burns in 7.8%, and elective surgery in 4.7%. The central nervous system involvement occurred in 56.3% of patients and was characterized as encephalopathy in 75%, seizures in 13.9%, non-epileptic myoclonus in 2.8%, and ischemic stroke in 8.3%. The magnetic resonance imaging, cerebrospinal fluid and electroencephalographic changes were unremarkable in encephalopathic patients. Neuromuscular disorders were diagnosed in 43.7%. Critical ill polyneuropathy was characterized in 57.1%, critical ill myopathy in 32.1%, demyelinating neuropathy in 7.2%, and pure motor neuropathy in 3.6%. Nerve and muscle pathological studies dismissed inflammatory abnormalities. The identification of these conditions has important economic implications and may change the critically ill patients' prognosis.

Guillain-Barré syndrome in the US Army.

Using the US Army's operational reporting system, the medical records of soldiers with Guillain-Barré syndrome were retrieved and compared with reported incidence figures from the 1976 national influenza immunization program. Military personnel sustained an incidence of disease approximatley 50% higher than unvaccinated civilians but less than 25% of the rate attributed to civilian vaccinees.

Guillain-Barré syndrome. Clinicoepidemiologic features and effect of influenza vaccine.

A study of the epidemiologic and clinical features of Guillain-Barré syndrome in the population of Olmsted County, Minnesota, over the 46-year period 1935 through 1980 was conducted through the centralized diagnostic index maintained at Mayo Clinic, Rochester, Minn. A total of 48 cases were identified, giving an age- and sex-adjusted incidence of 1.8 per 100,000 person-years. The rate increased over time from 1.2 in the interval 1935 through 1956 to 2.4 in the interval 1970 through 1980. Males were affected more than females (age-adjusted rates of 2.3 and 1.2, respectively). The rate increased with age from 0.8 in those under 18 years old to 3.2 for those 60 years and older. Antecedent infectious diseases were reported in 65% of the cases. Implications with regard to the incidence of Guillain-Barré syndrome associated with the A/New Jersey/76 (swine flu) vaccine are discussed.

Guillain-Barré syndrome in Larimer County, Colorado: a high-incidence area.

During the period 1981-1983, 19 cases of Guillain-Barré syndrome (GBS) occurred in residents of Larimer County, Colorado, for an incidence of 4.0 cases per 100,000 population per year, compared with 1.2 cases per 100,000 per year in 1975-1980 (p less than 0.05). The higher incidence of GBS in 1981-1983 may represent an unusual chance occurrence, since no patient characteristics or predisposing events could be found to explain the increase. Nevertheless, the findings demonstrate that over a period of as long as 3 years, the crude average annual incidence of GBS in a large, well-defined population may exceed by twofold the upper limit of the previously reported range (0.6 to 1.9 cases per 100,000 per year).

Results of the national surveillance for Guillain-Barré syndrome.

The preliminary results of the national surveillance for Guillain-Barré syndrome(GBS) are reported. In the first 6 months of 1978, 327 cases of GBS were reported to the Center for Disease Control (CDC). A statistically significant difference was observed between sex-specific attack rates, and a direct correlation also was observed between advancing age and increasing risk of GBS.

National surveillance for Guillain-Barré syndrome: January 1978-March 1979.

Between January 1, 1978, and March 31, 1979, 1,034 cases of Guillain-Barré syndrome (GBS) were reported to the Centers for Disease Control by the 1,813 American Academy of Neurology sentinel physicians who participated in the national GBS surveillance program. A direct correlation was observed between increasing age and the age-specific attack (incidence) rates. Based on the cases observed and the total US population, age-adjusted attack rates were statistically higher in males (0.52 per 100,000) than in females (0.40). Rates for whites were 0.44 and those for blacks 0.28 per 100,000; although the difference is statistically significant, uncertainties as to the true denominators by race preclude acceptance of these differences as valid. Sixty-seven percent, or 682 of the patients, reported that they had had an antecedent illness within 8 weeks before onset of GBS, and among them the peak period of onset of GBS was in the second week after the onset of the prior illness. There were also 52 patients (5%) who had undergone surgery and 45 (4.5%) who had received vaccinations, both within the 8 weeks before onset of GBS. However, the high proportions of antecedent illness in these groups (45% of those operated and 53% of those vaccinated) made attribution of GBS to the procedures tenuous. Risk of GBS in patients who reported receiving a swine influenza vaccination in 1976 was no greater than in those who reported that they did not receive this vaccine.

Incidence of Guillain-Barré syndrome during a nationwide oral poliovirus vaccine campaign.

Concomitant with a nationwide oral poliovirus vaccine (OPV) campaign in Finland in 1985, an unexpected rise in the number of patients hospitalized with the Guillain-Barré syndrome (GBS) occurred. An analysis based on hospital records covering a population of 1.17 million and 6 years revealed a significantly increased incidence of GBS coinciding with the OPV campaign. We carefully examined 10 cases with onset of GBS within 10 weeks after immunization. Only 4 patients could recall recent symptomatic infection, and we found no specific agent in these cases. Our study suggests that live-attenuated polioviruses may, like other infectious viruses, sometimes trigger the GBS.

Guillain-Barré syndrome in a Danish county.

In a retrospective population-based study, we reviewed clinical records of 51 patients with Guillain-Barré syndrome (GBS) during the years 1965 to 1982. The mean annual age-adjusted incidence rate was 1.14 per 100,000 people. Compared with reports from Israel, Danish and Swedish patients with GBS seem to have more extensive and more severe symptoms, although survival rate and recovery prognosis are probably the same.

A prospective study on the incidence and prognosis of Guillain-Barré syndrome in Emilia-Romagna region, Italy (1992-1993). Emilia-Romagna Study Group on Clinical and Epidemiological Problems in Neurology.

We performed a multicenter prospective study on the incidence and prognosis of Guillain-Barré syndrome (GBS) in the Emilia-Romagna region (3,909,512 inhabitants), Italy, over a 2-year period (1992-1993). The case finding method was based on a surveillance system including neurologic departments, all private and public general hospitals, and all practicing neurologists. We also reviewed all the ICD codes of hospital discharges. Ninety-four patients with GBS were identified, giving a mean incidence rate of 1.20 per 100,000 per year. Men/women ratio was 1.94. Incidence rate increased with age for both sexes. There were no significant incidence variations among seasons or months. Antecedent infections were reported in 59% of the patients. There was an overall good prognosis. After 6 months, 64% of patients had recovered and only 4% was still severely disabled. Acute mortality was 3%. Older age was the strongest predictor of poor outcome.

Guillain-Barré syndrome in the United States, 1978-1981: additional observations from the national surveillance system.

During the period January 1978-March 1981, 2,575 cases of Guillain-Barré syndrome (GBS) were reported by participating neurologists in the national GBS surveillance system. The incidence of GBS was highest in the 50- to 74-year-old age group, but a lesser peak was observed in persons aged 15 to 35. The frequencies of antecedent respiratory (43%) and gastrointestinal (21%) illness exceeded frequencies of such illnesses in the US population (10 and 0.8%, respectively), based on survey data compiled by the National Center for Health Statistics; the differences in these frequencies of illness were similar in all seasons of the year, in males and in females, and in persons less than 6, 6 to 16, 17 to 44, and greater than 44 years of age. Nineteen percent of adult patients for whom information was available (67% of the total) reported receiving an A/New Jersey influenza vaccine in 1976, a lower percentage than would be expected on the basis of a survey conducted in that year. The data suggest that persons who received this vaccine have not been at increased risk and may even have been at decreased risk of acquiring GBS during the period covered by this study.

Frequency of adverse reactions to hepatitis B vaccine in 43,618 persons.

PURPOSE: To determine the incidence of adverse reactions to hepatitis B plasma-derived vaccine. PATIENTS: Alaska natives (43,618) who received 101,360 doses of hepatitis B vaccine. METHODS: All adverse reactions, excluding transient fever, myalgia, or soreness lasting less than 3 days, were reported. An intradermal skin test was developed to test purported adverse reactions. Records of the entire population were reviewed for Guillain-Barré syndrome (GBS). SETTING: A statewide hepatitis B control program for Alaska natives. RESULTS: Possible adverse reactions occurred in 39 persons. The most frequent adverse reactions were myalgia/arthralgia lasting longer than 3 days (14), followed by skin rashes (eight) and dizziness (seven). Skin tests were performed on 13 persons and were positive in five. Six of the persons with negative skin tests and eight persons who did not undergo skin testing received additional doses of vaccine without any adverse reactions. No increased incidence of GBS was found in the vaccinees. CONCLUSION: Hepatitis B vaccine is safe and most adverse reactions are coincidental.

Antibodies to gangliosides and galactocerebroside in patients with Guillain-Barré syndrome with preceding Campylobacter jejuni and other identified infections.

The relationship between preceding infections and antibodies to glycolipids was investigated in 205 Japanese patients with Guillain-Barré syndrome (GBS). Serological evidence of recent Campylobacter jejuni (C. jejuni) infection was found in 45% of the patients, compared with 1% in healthy controls. In contrast, recent infection of cytomegalovirus (CMV), Mycoplasma pneumoniae (M. pneumoniae) and Epstein-Barr virus (EBV) was detected in only 5%, 2% and none of the patients, respectively. C. jejuni-associated GBS was more frequent in early spring than in other seasons. All stool specimens positive for C. jejuni isolation were obtained within 10 days after the onset of GBS symptoms. Of 13 C. jejuni isolates from GBS patients, 10 (77%) belonged to Penner serotype 19 (heat-stable, HS-19). Elevated titers of anti-GM1 antibody were found in 8 (80%) of 10 GBS patients whose C. jejuni isolates belonged to HS-19 and in none of those infected with non-HS-19 C. jejuni (P = 0.04), and in 49% of 92 patients with C. jejuni infection and 25% of patients without infection of C. jejuni, CMV, EBV, or M. pneumoniae (P = 0.0007). The frequencies of elevated antibody titers to GD1a, GD1b and GQ1b were also significantly higher in GBS patients associated with C. jejuni than those not associated with C. jejuni, CMV, EBV, and M. pneumoniae. GBS in Japan seems to be associated more frequently with C. jejuni and less frequently with CMV than in Europe and North America.