Idiopathic spinal cord herniation: value of MR phase-contrast imaging.

We report two patients with an idiopathic transdural spinal cord herniation at the thoracic level. Phase-contrast MR imaging was helpful in showing an absence of CSF flow ventral to the herniated cord and a normal CSF flow pattern dorsal to the cord, which excluded a compressive posterior arachnoid cyst.

Overproduction of proinflammatory cytokines imbalanced by their antagonists in POEMS syndrome.

The polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes (POEMS) syndrome is a rare multisystem disorder of obscure pathogenesis associated with osteosclerotic myeloma. Circulating levels of proinflammatory cytokines (tumor necrosis factor-alpha (TNF-alpha) interleukin-1 beta [IL-1 beta], IL-2, IL-6, and interferon-gamma [IFN-gamma]), anti-inflammatory cytokines (transforming growth factor beta 1 [TGF beta 1], IL-4, IL-10, and IL-13), the cytokine carrier protein alpha 2 macroglobulin, IL-1 receptor antagonist (IL-1ra), soluble TNF receptors (sTNFr) p55 and p75, and soluble IL-6 receptor (sIL-6r) were determined in 15 patients with POEMS syndrome and 15 with multiple myeloma. Patients with POEMS syndrome had higher serum levels of IL-1 beta, TNF-alpha, and IL-6 and lower serum levels of TGF beta 1 than did patients with multiple myeloma. Serum levels of IL-2, IL-4, IL-10, IL-13, IFN-gamma, alpha 2 macroglobulin, and sIL-6r were similar in both groups. IL-1ra and sTNFrs were increased in POEMS syndrome, but out of proportion to the increase of IL-1 beta and TNF-alpha. Serial evaluations in 1 patient showed that proinflammatory cytokine serum levels paralleled disease activity assessed by platelet count and neurologic involvement. Our results suggest that the manifestations of POEMS syndrome might be regarded as the result of a marked activation of the proinflammatory cytokine network (IL-1 beta, IL-6, and TNF-alpha) associated with a weak or even decreased (TGF beta 1) antagonistic reaction insufficient to counteract the noxious effects of cytokines.

Elevated levels of interleukin-1 beta (IL-1 beta) and IL-6 in serum and increased production of IL-1 beta mRNA in lymph nodes of patients with polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome.

To evaluate a possible implication of cytokines in the pathogenesis of polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) syndrome, we studied five consecutive patients with this condition, of which four had sclerotic bone lesions and four had multicentric Castleman's disease. Interleukin-1 beta (IL-1 beta) and IL-6 serum levels were determined in these patients (13 serum samples) and in patients with multiple myeloma (5) and Waldenström's macroglobulinemia (5). In situ hybridization of the relevant mRNAs was performed on lymph node specimens of two patients with POEMS syndrome who had Castleman's disease. Elevated serum levels of IL-1 beta (13/13 samples), and IL-6 (7/13 samples) were found in patients with POEMS syndrome. In the other patients, serum IL-1 beta was undetectable or slightly increased and IL-6 was elevated in a single patient with Waldenström's macroglobulinemia. Abundant IL-1 beta mRNA-producing cells were present in interfollicular spaces in the two tested patients, while IL-6 mRNA-producing cells were rare. We conclude that IL-1 beta and IL-6 serum levels may be chronically elevated in patients with POEMS syndrome, and that lymph node may be one site of IL-1 beta overproduction. These results are in keeping with the hypothesis that cytokines mediate systemic manifestations of POEMS syndrome.

Early weight loss and high serum tumor necrosis factor-alpha levels in polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes syndrome.

We studied tumor necrosis factor-alpha (TNF-alpha) levels in serial serum samples from 3 consecutive patients who had a complete form of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome and early weight loss. Serum TNF-alpha levels were compared with those of 10 patients with multiple myeloma (5) or Waldenström's macroglobulinemia (5). Elevated serum levels of TNF-alpha were found in the patients with POEMS syndrome (two- to eightfold increase in 10 of 11 samples) and not in those with other malignant plasma cell dyscrasias. These results are in keeping with the hypothesis of a role for nonimmunoglobulinic mediators in the pathogenesis of the POEMS syndrome, and are consistent with previous reports of TNF-alpha overproduction in inflammatory demyelinating neuropathies and cachectic states.

Late-onset familial amyloid polyneuropathy with the TTR Met 30 mutation in France.

Four unrelated French cases of familial amyloid polyneuropathy are reported. Clinical onset ranged from the sixth to the ninth decade. Sensory signs were predominant initially in the lower limbs; motor changes, and in one case autonomic involvement, appeared later. Amyloid disease was clinically limited to the peripheral nervous system. In two cases, there was no evidence of familial disease. DNA analysis was performed in these four patients and in two children of Patient 1. Restriction analysis of amplification products of exon 2 of the transthyretin gene was positive for the valine 30 to methionine mutation. These four unrelated patients live in different areas of France. Further studies are needed to determine whether these mutations have a common origin and whether they are related to the Portuguese mutation.

Giant gangliocytic paraganglioma of the filum terminale. Case report.

The clinical and pathological features of a giant cauda equina paraganglioma arising from the intradural filum terminale is described. Scattered mature large neurons characterized the tumor as a gangliocytic paraganglioma. Histologically, these neoplasms have considerable similarity with ependymoma and the diagnosis can be easily missed unless special techniques are employed.

[Painful legs and moving toes. Neuropathy caused by cytarabine]. / Jambes douloureuses et orteils instables. Neuropathie induite par la cytarabine.

A new case of painful legs and moving toes syndrome is reported in a patient with a neuropathy due to high-doses of cytosine arabinoside (Ara C). A few cases of this complication have been reported. The neuropathy was sensory, axonal, and incompletely regressive. Carbamazepine had a dramatic effect on pain and involuntary movements.

Pathological findings in three non-Japanese patients with the POEMS syndrome.

The pathological features of three European patients with plasma cell dyscrasia, osteosclerosis and a multisystem disorder, most frequent in Japan, that includes polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome), are reported. The material was obtained from biopsies (peroneal nerve, lymph node) and general autopsy, including hypophysis, in one case. The most salient findings were: peripheral nerve lesions, including both segmental demyelination and axonal degeneration, with so-called uncompacted myelin lamellae (UML); angiofollicular lymph node hyperplasia (AFLNH); and non inflammatory vascular changes. Though not specific, it appears that UML and AFLNH may be contributive findings in atypical cases of POEMS syndrome (incomplete forms, lack of underlying malignant plasma cell dyscrasia or circulating monoclonal immunoglobulin). Among the various autopsy findings we emphasize the skin thickening which was secondary to a hyaline sclerosis of the papillary dermis, and the presence in adenohypophysis of numerous cells showing positive reactions with the anti-alpha MSH antibody. Though immunological, vascular and hormonal disturbances have been implicated at the origin of several manifestations of the disorder, the pathogenesis of the POEMS syndrome remains obscure.