Visceral disseminated varicella zoster virus infection with brachial plexus neuritis detected by fluorodeoxyglucose positron emission tomography and computed tomography.

Varicella zoster virus (VZV) infection sometimes result in visceral disseminated VZV infection (VD-VZV), which is a fulminant disease featured by abdominal pain and the absence of skin lesions, particularly occurs in the immunosuppressive patients. Brachial plexus neuritis (BPN) is another rare type of VZV infection usually appears without blisters. Few diagnostic images of both VD-VZV and BPN-VZV have been reported. A 25-year-old woman receiving allogeneic hematopoietic stem cell transplantation (HSCT) for acute myeloid leukemia. Unexplained severe pain in the left upper extremity followed by severe stomachache, liver dysfunction and unconsciousness appeared on day 344 post-HSCT. Computed tomography (CT) showed left brachial plexus hypertrophy and edematous changes to the hepatoduodenal ligament, fluorodeoxyglucose positron emission tomography (FDG-PET) showed increased uptake in both lesions. Intravenous acyclovir therapy was started and successfully resolved all symptoms. Several days later, blisters appeared all over the body and positive VZV DNA from blood using polymerase chain reaction test was obtained. FDG-PET and CT may offer supportive findings for detecting or diagnosing blister-less VZV infectious diseases.

Cerebrospinal fluid profile and seroprevalence of antiganglioside reactivity in patients with neuralgic amyotrophy.

Neuralgic amyotrophy (NA), also known as acute brachial plexitis, is postulated as an autoimmune pathogenesis. In a well-defined cohort of patients with NA, we analyzed the cerebrospinal fluid (CSF) profile and the prevalence of antiganglioside antibodies. Patients with Varicella zoster-associated radiculitis and healthy blood donors served as controls. An abnormal routine laboratory CSF profile was found in 29% of those with NA, mostly showing a disruption of the blood-brain barrier. Antibodies predominantly from the immunoglobulin M (IgM) isotype against at least one human ganglioside were detected in 36% of sera from patients with NA but in only 2% of controls. An NA-specific reactivity pattern was not detected, and there was no significant association with clinical or CSF parameters. This suggests that the seroprevalence of antiganglioside autoantibodies in patients with NA is nonspecific.

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.

Intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy for motor impairment of neuralgic amyotrophy: clinical observations in 10 cases.

BACKGROUND: Neuralgic amyotrophy (NA) is a distinct peripheral nervous system disorder characterized by attacks of acute neuropathic pain and rapid multifocal weakness and atrophy unilaterally in the upper limb. The current hypothesis is that the episodes are caused by an immune-mediated response to the brachial plexus, however, therapeutic strategies for NA have not been well established. METHODS AND RESULTS: We retrospectively reviewed 15 case series of NA; 10 of the 15 patients received intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy (MPPT) and 9 of these 0 patients showed clinical improvement of motor impairment. CONCLUSION: Our clinical observations do not contradict the possibility that IVIg with MPPT may be one of the potential therapeutics for NA, however the efficacy remains to be established. Further confirmatory trials are needed in patients with various clinical severities and phases of NA. Further basic research and confirmatory trials should be performed to survey the efficacy of such immunomodulation therapy for NA.

Four cases of anti-ganglioside antibody-positive neuralgic amyotrophy with good response to intravenous immunoglobulin infusion therapy.

Neuralgic amyotrophy (NA), which is an idiopathic disorder in the peripheral nerves, is characterized by an acute onset of unilateral pain in the proximal limbs followed by muscular weakness and wasting. Some cases of NA are thought to be related to immune pathogenic disorders such as Guillain-Barré syndrome (GBS). We report the case of four patients with NA who were positive for anti-N-acetylgalactosaminyl GD1a (anti-GalNAc-GD1a) antibodies, had a preceding infection, and showed a good response to intravenous immunoglobulin infusion therapy. Anti-ganglioside antibodies, especially the anti-GalNAc-GD1a antibody, may be a useful marker for predicting response to immune therapy.

Clinical and pathophysiological concepts of neuralgic amyotrophy.

Neuralgic amyotrophy--also known as Parsonage-Turner syndrome or brachial plexus neuritis--is a distinct and painful peripheral neuropathy that causes episodes of multifocal paresis and sensory loss in a brachial plexus distribution with concomitant involvement of other PNS structures (such as the lumbosacral plexus or phrenic nerve) in a large number of patients. The phenotype can be limited or extensive and the amount of disability experienced also varies between patients, but many are left with residual disabilities that affect their ability to work and their everyday life. Both idiopathic and hereditary forms exist. The latter form is genetically heterogeneous, but in 55% of affected families, neuralgic amyotrophy is associated with a point mutation or duplication in the SEPT9 gene on chromosome 17q25. The disease is thought to result from an underlying genetic predisposition, a susceptibility to mechanical injury of the brachial plexus (possibly representing disturbance of the epineurial blood-nerve barrier), and an immune or autoimmune trigger for the attacks. The precise pathophysiological mechanisms are still unclear; treatment is empirical, and preventive measures are not yet available. This Review provides an overview of the current clinical and pathophysiological concepts and research topics in neuralgic amyotrophy.

[A case of neuralgic amyotrophy with antiganglioside antibody].

A 54-year-old-man experienced pain from his left shoulder to his left arm and had difficulty in lifting his arm after a febrile episode. Three weeks after the onset, he was admitted to our hospital. Neurological examination demonstrated weakness and atrophy of the left deltoid muscle. Deep tendon reflexes were normal and no pathological reflexes were elicited. CSF total protein was slightly increased. The occurrence rate of F-waves was decreased in the left upper limb. Magnetic resonance imaging (MRI) study of the cervical cord and brachial plexus with and without Gadolinium infusion showed no abnormalities. Serological study showed that IgM anticytomegalovirus antibody was positive, and that serum IgM anti-GalNAc-GD1a antibody and IgM anti-GM2 antibody were positive. Symptoms were improved after treatment with mecobalamin, 1.5mg/day. This case was considered neuralgic amyotrophy after cytomegalovirus infection. The antiganglioside antibodies may play some role in its pathogenesis.

Wartenberg's migrant sensory neuritis.

We describe a patient with the sudden onset of a painful, purely sensory, mononeuritis multiplex. Investigations showed no evidence for any underlying systemic condition. A nerve biopsy showed fascicular wallerian degeneration with perineurial thickening, inflammatory cells, and immunoglobulin G (IgG) deposition. His painful sensory deficits persisted, with no improvement after treatment with prednisone. The clinical characteristics in this case were very similar to those originally described by Wartenberg, and subsequently by other investigators. The investigations in our case strongly suggest that there may be an underlying immune pathogenesis for cases of Wartenberg's migrant sensory neuritis.

Blood lymphocytes are sensitized to branchial plexus nerves in patients with neuralgic amyotrophy.

The percentage of lymphocytic subsets in the blood of cases with neuralgic amyotrophy (NA), and the proliferative response of blood lymphocytes cultured with different nerve extracts, obtained from normal subjects at postmortem, were examined in 6 patients with NA and in 18 age-matched controls with shoulder pain not related to NA. Most (5/6) NA patients had decreased CD3 values and increased CD4/CD8 ratios due to a decreased of the CD8 subset. Lymphocytes of NA patients increased their blastogenic activity in cultures with nerve extracts from different brachial plexus nerves and its branches, but not in cultures with extracts of sacral plexus nerves. Cultures did not respond to nerve extracts in any of the control cases, although mitogenic activity was similarly elicited in cultured lymphocytes stimulated with phytohemagglutinin in both control cases and NA patients. These results suggest that NA is probably an immune mediated disease.

Recurrent brachial plexus neuropathy and giant cell arteritis.

A 73-year-old women presented with a recurrent form of sporadic brachial plexus neuropathy, the so-called Parsonage and Turner syndrome. This diagnosis is based on clinical and electromyographic findings. Interestingly a biopsy of the temporal artery demonstrated a giant cell arteritis. The clinical picture started 2 weeks after an upper respiratory tract illness. The possible viral etiology of giant cell arteritis is considered. We think an immunological rather than ischemic disturbance may have caused the recurrent brachial plexus neuropathy. This case report suggests that giant cell arteritis be considered in the investigation of the Parsonage and Turner syndrome.

[A case of "neuralgic amyotrophy" with elevated serum antibody titer against Borrelia burgdorferi].

A 39-year-old man experienced an abrupt onset of right back pain. The pain improved spontaneously, but weakness of the right upper extremity developed. The weakness deteriorated during the next month, and he was admitted to our hospital. Neurological examination disclosed impairment of superficial sensation in his right upper extremity. Blood examination showed no abnormal data. The cerebrospinal fluid was normal. Neuroradiological findings were also negative. Electrophysiological examinations were normal except for needle electromyographic findings of the right upper extremity, which showed neurogenic patterns of moderate degree. Those findings suggest neuralgic amyotrophy. However, examining the serum sample significantly elevated levels of antibody titers against Borrelia burgdorferi were observed, and we suspected that his illness was Lyme disease. He recalled, however, no arthropod bite. Neuralgic amyotrophy is a syndrome which takes a characteristic clinical course. It includes some heterogeneous disorders. On the other hand, Lyme disease, a tick-transmitted spirochetal illness, occurs in stages, with remissions and exacerbations and different clinical manifestations at each stage. The neurological abnormalities include aseptic meningitis, encephalitis, cranial neuritis, motor and sensory radiculitis, and myelitis in various combinations. They can be diagnosed serologically. However, it is possible that elevation levels of the antibody titers mean nonspecific damages of peripheral nerves. Further study is necessary to decide whether cases like ours suffer from so-called Lyme disease or not.