Depression of Ca2+ influx in complement C5a-stimulated neutrophils by calmodulin inhibitors

Role of Ca2+/calmodulin complex in intracellular Ca2+ mobilization in neutrophils has not been clearly elucidated. In this study, effects of chlorpromazine, trifluoperazine and imipramine on the intracellular Ca2+ mobilization, including Ca2+ influx, in C5a-activated neutrophils were investigated. Complement C5a-stimulated superoxide production and myeloperoxidase release in neutrophils were inhibited by chlorpromazine, trifluoperazine and imipramine, except no effect of imipramine on myeloperoxidase release. A C5a-elicited elevation of (Ca2+)i in neutrophils was inhibited by chlorpromazine, trifluoperazine, imipramine, staurosporine, genistein, EGTA, and verapamil but not affected by pertussin toxin. The intracellular Ca2+ release in C5a-activated neutrophils was not affected by chlorpromazine and imipramine. Chlorpromazine and imipramine inhibited Mn2+ influx by C5a-activated neutrophils. Thapsigargin-evoked Ca2+ entry was inhibited by chlorpromazine, trifluoperazine, imipramine, genistein, EGTA and verapamil, while in the activation process of neutrophils. The depressive action of calmodulin inhibitors on the elevation of cytosolic Ca2+ level in C5a-activated neutrophils appears to be accomplished by inhibition of Ca2+ influx from the extracellular medium.

A Case of Thrombotic Thrombocytopenic Purpura(TTP), Presenting Multiple Gross Infarction

Thrombotic thrombocytopenic purpura (TTP) is an uncommon disorder, frequently fatal, characterized by a pentad of thrombocytopenia, microangiopathic hemolytic anemia, fluctuating neurologic symptoms, fever, and renal disease. TTP is characterized by widespread noninflammatory thrombotic occlusions of arterioles and capillaries with material containing fibrin and platelets. Generally, gross infarct is rare in the patient with TTP. We present a case of TTP with multiple gross infarcts on brain CT scan. A 53-year-old-man was presented with left hemiparesis and confused mentality. On laboratory study, he had microangiopathic hemolytic anemia & thrombocytopenia. Brain CT showed multiple gross infarct areas in the right basal ganglia, right parietal lobe and right cerebellar hemisphere. When managing the patient with acute stroke syndrome, we should consider TTP as a diagnostic possibility for early proper treatment, even if CT scan of brain show multiple gross infarctions.

Pathophysiologic Approach by Blink Reflex and EMG Studies in Essential Blepharospasm Comparison with Other Facial Involuntary Movement

Background and objectives: Blepharospasm (BS) is best categorized as focal dystonia, but the biochemical and neuroanatomical mechanisms are poorly understood. We performed this study in order to postulate the pathophysiologic mechanism of essential BS, using blink reflex test and EMG studies. METHODS: We studied 24 patients with essential BS and 51 normal adults. Blink reflex tests and EMG on orbicularis oculi muscle were performed in all patients. We evaluated our electrophysiological data, comparing with those obtained from other studies, in which bulbocavernosus reflex, H-reflex, and T-reflex tests were done. We also compared our EMG data with those of hemifacial spasm and facial myokymia in other studies. Results: 1. Rl response latency of blink reflex test in 24 patients with essential BS was not changed, but R2 latency in the papient group was significantly shortened, comparing with those of normal control group. These results can be explained by overexcitability of the interneuron with polysynaptic pathway. Another supporting evidence is the fact that the latency of polysynaptic bulbocavernous reflex test in upper motor neuron lesion is shorter than normal control, although the latency of monosynaptic H-reflex and T-reflex test are not changed. 2. EMG on orbicularis oculi muscle in patients with essential BS showed spontaneous MUPs at irregular intervals at rest, while those in patients with hemifacial spasm and facial myokymia, known to be caused by overexcitability of facial nucleus, showed spontaneous MUPs at regular intervals. EMG in patients with essential BS, during the period of spasm, showed relatively long brief bursts at irregular intervals from 30 to 100 ms, lasting inconstant duration ranging from 30 to 300 ms. CONCULSIONS: It is suggested that the pathophysiology of essential BS is overexcitability of interneuron due to disinhibition in inhibitory interneuron, and that EMG findings of essential BS are remarkably different from those of hemifacial spasm and facial myokymia.

A case of Myasthenia Gravis associated with Premature ovarian failure and thyroid autoanibodies

Myasthenia gravis (MG) is regarded as autoimmune disorder of neuromuscular transmission and apparently is due to formation of humoral antibody that cross-link to acetylcholine receptor at skeletal muscle end plate. Patients with autoimmune diseases often have circulating antibodies to a variety of different autoantigens and show a variety of manifestations of autoimmune diseases in more than one organ. Therefore, MG patients frequently have manifestations of autoimmune diseases such as thyroid disease, rheumatoid arthritis and systemic lupus erythematosus. But MG in association with premature ovarian failure (POF) rarely has been described. A case of MG with POF and thyroid autoantibodies was experienced and presented with a brief review of literature.

A Case of Paramyotonia Congenita

Paramyotonia congenita (PMC), an autosomal dominant non-progressive muscle disorder, is characterised by cold-induced stiffness followed by muscle weakness. The weakness is considered to be caused by a dysfunction of the sodium channel in muscle fiber. We report a 37-year-old male patient with PMC, complaining of episodic myotonia and motor weakness on cold exposure. In this patient, we performed clinical and neurological examination, electrophysiologic examination and muscle biopsy. On electrophysiologic study, needle EMG showed spontaneous myotonic discharges at room temperature but disappeared after cooling. Amplitude of compound action potential in abductor pollicis brevis muscle decreased significantly after cooling the tested extremity. Muscle biopsy showed a minimal variation of muscle fiber diameters, internal nuclei, chained nuclei, occasional atrophic fibers in vastus lateralis muscle. His mother, his son, three of six siblings, and five of eleven nephewes are affected with same symptomes.

A Study of Blink Reflex and Direct Facial Nerve Stiimulation in Normal Korean Adult

The blink reflex is an electrically induced glabella response that has long been used in clinical neurology. The blink reflex is now known to be a polysynaptic reflex with an afferent arc through sensory fibers of the trigerminal nerve and with an c,fferent arc through the motor fibers of the facial nerve. This study was performed to determine the relationship of latency and amplitude to ethnic groups sex and age and to obtain the normal data of korean in the blink reflex(BR) and direct facial nerve stimulation test(DFNS). BR and DFNS were recorded in 103 normal korean adults that consisted of 52 men and 51 women and the age ranged 22 to 77 years. The results are as follows: (1) the Rl wave was more stable than R2 and contralateral R2 wave, (2) there seerns to be no significant difference of latency and amphtude between ethnic groups, (3) the latency of Rl wave was shorter and the amplitude of CMAP was larger m men compared with women and (4) the latency of Rl wave was increased by aging.

The Pathophysiologic Approach and Usefulness of Conventional Blink Reflex Test and Electromyography in Hemifacial Spasm

A series of 61 cases of primary hemifacial spasm have been evaluated by means of blink reflex and needle electromyography and their result compared with unaffected site. The R1 and R2 response and late acti-ity. After activity in blink reflex. And electrical activity in EMG at rest were observed. The results of study were as in the followings: 1) The arnplitude of large R1 and R2 responses were increased in affected side in contrast to unaffected side. 2) late activity(72.2%) and after activity(83.6%) were observed in only affected side at rela-ti-ely Iong duration, as well as regular interval spontaneous repetivive discharges in EMG at rest. This result of large R1 and R2 responses could be compatible with ephaptic ransmission, but late activity, after activity, and spontaneous repetitive MUPs could be explained hyperexcitability of the facial motor nucleus rather than autoexcitation of peripheral facial nerve. Additionally the authors thought that the use of conventional blink reflex as well as specialized blink reflex tests could be very useful for the diagnosis of hemifacial spasm and for the evaluation of it's course.

Two Cases of Avellis Syndrome

Avellis syndrome is a clinical disorder characterized by ipsilateral paralysis of palate, larynx, and vocal cord paralysis and contralateral hemianesthesia. The syndrome is usually due to lesion of medulla oblongata. Involeved structures are nucleus ambiguus, lateral spinothalamic tract, and descending sympathetic fibers. We present 2 cases of Avellis syndrome with MRI findings, which showed infarction of right-sided upper medulla oblongata.