Hyperekplexia: a non-epileptic startle disorder.
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OBJECTIVE: To create an experimental organ donor model and validate it by ultrasound evaluation of intracranial blood flow velocity. MATERIAL AND METHOD: Ten white New Zealand rabbits were assigned to either a control group or an experimental group. Brain death was induced in the experimental group by way of severe cranial hypertension. A diagnosis of brain death was based on physical examination and velocity of intracranial blood flow determined by transcranial doppler ultrasonography. Physical and ultrasound examinations were performed on all animals at baseline and at the time of the experiment. RESULTS: Physical examination and intracranial blood flow velocity were normal in the control group animals at baseline and during the study. The findings were also normal for the experimental group animals at baseline. After provocation of intracranial hypertension, we observed signs of brain death (absence of response to pain stimulus in one cranial par absence of brainstem reflexes, and apnea) in the experimental animals, and the velocity of flow in arteries at the base of the skull acquired the waveform known as sharp systolic peaks, which are characteristic of cerebral circulation failure. CONCLUSIONS: The results of physical examination and ultrasonography in this study provide adequate validation of this experimental model of brain death and demonstrate that transcranial doppler ultrasonography is useful for diagnosing brain death in the rabbit.
In rats of GC strain bred for predisdposition to cataleptic freezing, a significant negative correlation between the duration of freezing and the level of prepulse inhibition (PPI) of the startle reflex, has been found. Besides, in a group of GC rats specific by their "nervousness" and jumpiness, there was also a negative correlation between the duration of freezing and the habituation to the startle reflex. None of this correlation have been found in Wistar rats. Since impairment of the PPI and habituation of the startle reflex is considered to be characteristic of schizophrenia, it is believed than cataleptic freezing in the GC rats may be used as a model of schizophrenic psychopathology.
To investigate excitability of spinal neural function during stretching excises in patients with cerebrovascular disease (CVD), H-reflex was analyzed before, during and after 1 min. continued stretching of the affected arm. 10 hemiparesis patients with hypertonus and hyperreflex, mean age of 53.2 years were tested. H-reflex was recorded from the abductor pollicis brevis on the affected side after stimulation of median nerve in supine position. The persistence, amplitude and amplitude ratio of H/M during stretching were lower than those before and after in the patients with moderately increased muscle tonus. In patients with slightly and markedly increased, H-reflex was same before, during and after continued stretching. It is suggested that excitability of spinal neural function during 1 min. continued stretching was inhibited in the patients with moderately increased muscle tonus caused by CVD.
Clinical examination and conduction velocity measurements in peripheral nerves have been done on 56 patients with non-Hodgkin's lymphomas of high malignancy (NHL). It is suggested that: the evaluation of the influence of chemotherapy on peripheral nervous system (PNS) is possible by means of systematic neurological and electroneurographic studies; electroneurographic assessment is most important in the diagnosis of early subclinical stages of peripheral neuropathy; toxic influence of CBVPM/AVBP protocol on PNS is greater than CHOP schedule; impairment of PNS due to chemotherapy is reversible in patients during complete remission (CR).
Andermann syndrome is characterized by agenesis of corpus callosum, anterior horn cell disease, a mixed sensory and motor neuropathy, and facial dysmorphism, and is inherited as an autosomal recessive trait. A 7-month-old boy was admitted with developmental retardation. Head control was not gained and he could not sit. He had high arched palate, elongated facies and large angle of the mandible, which were compatible with the Andermann syndrome. Moderate hypotonicity and absent tendon reflexes were also noted. Serum creatine kinase level was normal. Magnetic resonance imaging of the brain showed agenesis of the corpus callosum. Electromyographic examination revealed the presence of both sensory and motor neuropathy. The patient was diagnosed as having the Andermann syndrome according to the clinical and laboratory findings and he is reported due to rare presentation.
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Analysis of low-frequency (0.01 to 0.16 Hz), sinusoidal rotary chair responses of 24 patients with bilateral caloric weakness revealed a wide range of vestibulo-ocular reflex (VOR) impairment. The rotational stimuli presented allowed characterization of the degree of VOR impairment from severe to no impairment even in patients with absent responses to ice water caloric irrigation. Five patterns of responses were described, which helps to explain the lack of correlation often seen between caloric and rotary chair test results, especially when rotational testing does not extend down to at least 0.02 Hz. Bilateral caloric weakness was confirmed in 79% and 71% of patients by test frequencies of 0.01 and 0.02 Hz. Rotational stimuli of 0.04, 0.08, and 0.16 Hz gave notably poorer performance with 54%, 50%, and 29% confirmation.
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In 53 patients in different periods of brain strokes the authors studied the functional state of spinal centers by means of registration of H-reflex and F-wave of the skeleton muscles. In spite of the stroke character a marked increase of excitation of the spinal centers was observed, it being especially significant in the subacute period of the disease. The increase of H-reflex amplitude was maximal in extremities, contralateral to the focus of the lesion, but it was observed on the lesion side as well. The patients with cerebral coma in the acute stage of the stroke demonstrated a descrease of relation between central and motor responses up to 12--13%, it gradually increased as the coma ceased. The latent period of F-wave on the both extremities was reliably lower than the control indices along the whole period of the study. The authors show the significance of registration in the dynamics of the muscle evoked potentials for topical diagnosis of the cerebral vessel process and for effective control of the administered therapy.
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We evaluated the effect of prolonged wrist extension on H reflex in the flexor carpi radialis (FCR) muscle and tendon jerk (T) reflex in the biceps brachii (BB) muscle of 17 chronic hemiplegic patients. H reflex of the FCR and T reflex of the BB were assessed every 5 minutes within 20 minutes during prolonged wrist extension and post-20 minutes after the extension. As a result, H reflex in the FCR was reduced by passive wrist stretch in 82% of the spastic limbs. The effect was larger in the higher spastic group. In 45% of the spastic limbs, T reflex in the BB also was reduced by passive wrist stretch. The inhibitory effects had a tendency to strengthen in accordance with the grade of muscle tone. We considered from these results, prolonged wrist extension generated inhibitory projections via probably group II afferents of the FCR in the homonym and in the transjoint in spastic limbs.