1/10. urticaria and lip fasciculation may be prodromal signs of brain malignancy.The association of urticaria and cancer usually is seen with lymphoreticular system malignancies. Recalcitrant intra-nostril pruritus has been associated with fourth-ventricle tumors of the brain. Rarely, urticaria has been described with cancer of the lung, usually small-cell adenocarcinoma. We describe a girl who suffered with chronic urticaria for 3 months before lip fasciculation began to be observed. CT scan revealed a brain tumor adjacent to the cerebellum, which was diagnosed as astrocytoma grade II. Because of the location, the tumor was not operable, but after one course of radiotherapy, both the urticaria and lip fasciculation disappeared.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
2/10. role of macrophages in onion-bulb formation in localized hypertrophic mononeuritis (LHM).A unique pathogenetic process for onion-bulb (Ob) formation is disclosed with disclosed with immunohistochemistry and electron microscopy. biopsy of a swollen segment of tibial nerve from a 42 year-old white female histologically demonstrated diffuse and angiocentric lymphocytic infiltrate in both endo- and perineurium with occasional lymphofollicular formation. Extensive Ob formation of nerve fibers was most striking with or without associated lymphocytes. axis-cylinders were intact in the majority of Ob. Immunocytochemically, Ob are composed of alternately laminated leaflets of schwann cells (S100 ) and mononuclear macrophage (HAM56 /LeuMl /muramidase ) processes but no perineurial (EMA ) cells. Immunohistochemical evidence of antigen presentation (HLA-DR/LN3 /Ia ) was confined to macrophages. Electron microscopy insinuates that intricate interactions between macrophages and schwann cells exists. Putative inhibition of remyelination along with proliferation of schwann cells most probably is secondary to the effects of macrophages secretory products. No direct participation of B or T lymphocytes was detected in Ob. Thus, modified macrophages may emit a factor for concomitantly promoting proliferation of schwann cells and an enzyme for myelin breakdown. In addition, only a few macrophages could be detected in some Ob and could be easily overlooked or misinterpreted as "vacuolated fibroblasts", if no immunohistochemical correlation is made, as modified macrophages making the external leaflets of Ob are more vacuolated.- - - - - - - - - - ranking = 5keywords = cell (Clic here for more details about this article) |
3/10. Hypertrophic mononeuritis clinically presenting with painful legs and moving toes.A 40-year-old woman presented with progressive lower leg pain and spontaneous toe movement. The EMG showed a posterior tibial nerve mononeuropathy and continuous myokymic discharges in posterior tibial-innervated muscles. The MRI revealed a markedly enlarged posterior tibial nerve. Toe movements and myokymia were unaffected by the proximal transection of the lesion but ceased abruptly when the distal end of the fusiform "tumor" was resected, suggesting that spontaneous electrical foci may have been located along the nerve lesion. The markedly enlarged nerve segment contained edematous, swollen fascicles with marked Schwann cell onion-bulb lesions and angiocentric, lymphocytic, and lymphofollicular infiltration. This nerve lesion is an example of a newly recognized entity called hypertrophic mononeuritis.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
4/10. From benign fasciculations and cramps to motor neuron disease.Fasciculations and cramps may occur in motor neuron disease or as part of a more benign syndrome. A man with apparently benign fasciculations and cramps for 4 years developed progressive muscle weakness and wasting. Such a previously undocumented evolution of benign fasciculations and cramps to motor neuron disease may further implicate anterior horn cell dysfunction in the pathogenesis of muscle fasciculation-cramp syndromes.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
5/10. mercury intoxication simulating amyotrophic lateral sclerosis.A 54-year-old man had a syndrome resembling amyotrophic lateral sclerosis after a brief but intense exposure to elemental mercury. The syndrome resolved as his urinary mercury levels fell. mercury toxicity must be considered not only in individuals with recent anterior horn-cell dysfunction but also with otherwise unexplained peripheral neuropathy, tremor, ataxia, and a gamut of psychiatric symptoms including confusion and depression.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
6/10. poliomyelitis-like illness after acute asthma (Hopkins syndrome): a histological study of biopsied muscle in a case.A case of Hopkins syndrome is presented. The patient was a 4-year-old boy who developed weakness of the right leg 2-3 days after a mild asthmatic attack. Needle electromyography revealed fasciculation discharges in the right gastrocnemius muscle. A histological study of the biopsied right quadriceps femoris muscle revealed scattered atrophic fibers, indicating lesions in the anterior horn cells of the spinal cord. This is the first reported case of Hopkins syndrome including muscle pathology.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
7/10. serum of Isaacs' syndrome suppresses potassium channels in PC-12 cell lines.Blockage of K channels in nerve terminals by immunoglobulin is the speculated pathomechanism of Isaacs' syndrome. Using patch-clamp technique (whole-cell clamp), we investigated the effects on K current of serum taken from 2 patients with Isaacs' syndrome employing the clonal cell line PC-12. The addition of a patient's serum to the perfusion solution had little effect on the K current of P-12 cells. In contrast, K current was reduced by 25-80% when cells were cultured for 3-6 days with 2% serum as compared to control serum values. Suppression of the K current appears to develop gradually over the period of culture. Our results suggest that the pathomechanism of Isaacs' syndrome is caused by K channel suppression via a humoral factor(s) in the serum, which subsequently induces nerve hyperexcitability.- - - - - - - - - - ranking = 8keywords = cell (Clic here for more details about this article) |
8/10. antibodies to potassium channels of PC12 in serum of Isaacs' syndrome: Western blot and immunohistochemical studies.We investigated the pathophysiology of nerve hyperexcitability in a patient with Isaacs' syndrome, who had typical clinical and electromyographic features and responded to plasma exchange. immunoblotting and immunohistochemistry studies showed that antibodies from this patient reacted with the lysate of a neuronal cell line (PC12). In Western blots, constituents of the patient's serum, particularly immunoglobulin m, reacted with proteins of approximately 50 and 18 kDa, whereas the control serum did not. A cross-linking study with alpha-dendrotoxin (7 kDa) showed a 57 kDa protein-peptide complex. immunohistochemistry showed that the patient's serum reacted with pc12 cells and human intramuscular nerve axons. Our findings indicate that in Isaac's syndrome nerve hyperexcitability is the result of the immunological involvement of the voltage-dependent potassium channels located along the distal motor nerve or at the nerve terminal.- - - - - - - - - - ranking = 2keywords = cell (Clic here for more details about this article) |
9/10. ECG-related fasciculation potential.We report the observation of an unusual fasciculation potential (FP). This FP was time-locked to the electrocardiogram (ECG) in 87% of the discharges. The motor unit action potential (MUAP) of the FP was also elicited as h-reflex. As mechanism we propose spindle afferent discharges mechanically triggered by the arterial pulse, i.e. a reflex fasciculation. We suggest that such a heartbeat-related mechanism could explain synchronous fasciculations described by Norris (Arch. Neurol., 1965, 13: 495-500).- - - - - - - - - - ranking = 0.013719522559593keywords = spindle (Clic here for more details about this article) |
10/10. Continuous muscle fibre activity: a case treated with acetazolamide.A case is reported of the continuous muscle fibre activity syndrome, which includes a group of disorders characterised by sustained motor unit activity due to hyperactivity of peripheral nerve motor axons. In this patient the muscle stiffness and myokymic movements were successfully treated with acetazolamide, which acts as a membrane stabiliser either by blockade of chloride and bicarbonate membrane transport or by producing kaliuresis and raising the transmembrane potential by decreasing extracellular potassium.- - - - - - - - - - ranking = 1keywords = cell (Clic here for more details about this article) |
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