World Journal of Clinical Medicine and Pharmacy01

World Journal of Clinical Medicine and Pharmacy01

In China, the pathogenicity of the PKD pedigree is located in the 10.2 cM region between D351314 and D3S1265 of 3q28-29, which is a new pathogenicity gene of PKD.

1.2 ion channel disease PKD onset time is short, intermittent completely normal, the same patient each attack have similar triggering factors, and sodium channel block as the main mechanism of antiepileptic drugs such as carbamazepine and topiramate and so on Have a good effect, these phenomena support the PKD may be an ion channel disease point of view. Margari and other studies found that PKD patients with somatosensory evoked potentials and motor evoked potentials, combined with the clinical manifestations of PKD, PKD and research that the brain and muscle over-excited about, and this is likely to be the result of ion channel disorders. Thiriaux and other observed in the same patient in different age groups of PKD onset of symptoms may be different growth and development stages of various ion channel subunit expression differences. The current theory of ion channel disease has not been directly supported by empirical evidence.

1.3 abnormal function of the basal ganglia due to PKD attack can occur dystonia posture, dance-like movements, hand, foot and Xu Xu, such as extrapyramidal symptoms of extrapyramidal seizures after the general non-EEG abnormalities, seizures also unconscious Change, many scholars believe that PKD should be extrapyramidal diseases, and abnormal function of the basal ganglia, some studies also support this view. Zhou et al. Used functional magnetic resonance imaging (fMRI) to locate the abnormal regions of brain function in primary PKD. Compared with the control group, the amplitude of low-frequency fluctuation (ALFF) of bilateral nucleus and left posterior central gyrus increased, Suggesting that cortical - striatum - globus pallidus - thalamic loop abnormalities may be related to the pathophysiology of idiopathic PKD process. Joo and other studies using single photon emission computed tomography (SPECT) observed in the posterior region of bilateral caudate nucleus low perfusion, that this phenomenon and PKD extrapyramidal symptoms have a certain association.

1.4 cortical and spinal cord insufficiency PKD abnormal movement may be the cortex and spinal cord on the peripheral movement inhibition of the weakened function. (TMS) method for PKD patients were tested and found that despite the onset of control with carbamazepine, patients still have cortical inhibition and spinal cord suppression pathway abnormalities, showed short-term intracortical inhibition (SICI) abnormalities , But the normal cortical silent period, which and primary dystonia and some types of TMS abnormal pattern of epilepsy, but also indirectly proved PKD is a relatively independent disease. TMS results in patients with PKD demonstrated the presence of abnormal glycine and gamma-aminobutyric acid (GABA) can inhibit. Shin and other patients with primary PKD peripheral inhibition (SI) determination. SI refers to the activity of neural network in the peripheral region of the neural activity is inhibited, is a physiological mechanism to help select the most appropriate neuronal activity, is the necessary mechanism of the motor system. (MEP) was used to measure SI. When the index finger flexed, the amplitude of MEP from the little finger muscle was significantly inhibited. There was no significant difference between the experimental group and the control group. But after the flexion movement of the index finger, the motor evoked potential (MEP) In the control group, the MEP amplitude of the muscle of the little finger was increased, but no similar phenomenon was found in the experimental group. PK phenomenon in patients with SI extended to the movement after the end of the inhibition seems to weaken the view does not match, this may be the onset of the body to prevent the onset of unpredictable effect, which is the adaptation of PKD phenomenon, rather than PKD primary performance . In addition Frasson so that the level of the spinal cord and cortical somatosensory inhibition of the weakening of the process can be caused by the movement of abnormal sensory connection, and then motor abnormalities.