Cheiro-oral Syndrome: A Clinicoradiological Review of 10 Patients

BACKGROUND AND OBJECT: Cheiro-oral syndrome (COS) is characterized by a sensory disturbance in the unilateral hand and ipsilateral mouth corner. It is usually due to a lesion in the parietal cortex, thatamocortical projections, thalamus, or rarely brain stem. However, the syndrome is relatively unknown and rarely mentioned in most neurological textbooks. We presented ten cases of COS with a review of the clinical symptoms and signs and the neuroradiological methods used to demonstrate the responsible site. METHODS AND RESULTS: We studied 10 patients with stroke who showed restricted sensory disturbance on the one hand and ispilateral mouth. The study forms consisted of clinical manifestaion, neurological examination, electrophysiological, and neuroradiologic studies. Computed tomography and/or magnetic resonance imaging identified lesion in the thalamus in 5, brain stem in 3, and corona radiata in 1 patient. But, the anatomical responsible site for one case was not founded. Infarction had occurred in nine cases and hemorrhage in one. Seven of the 10 patients showed sensory disturbances restricted to the perioral area, hands, fingers when they were first examined; the remaining patients complained more diffuse sensory disturbances at first, but it had become restricted to perioral and fingers, usually within 2-3 weeks. The durations of symptom varied from 5 days to more than 15 months and these symptoms were improved within 2-3 weeks to 4 months in treated patients. CONCLUSION: When the symptoms and signs of the COS were presented, especially if a history of migraine is lacking, neuroradiological methods such as CT or MRI should be undertaken to localize and diffentiate the nature of lesion.

Prognostic Value of Elactrophysiologic Tests in Bell's Palsy

Bell's palsy is a relatively common, unilateral facial paralysis of unknown etiology. The purpose of this study was to evaluate the prognostic value of several electrophysiologic tests in Bell's palsy. Blink reflex (BR), side-to-side compound muscle action potential (CMAP) amplitude comparison, and side-to-side nerve excitability test (NET) threshold differ once have been studied during the first 2 weeks in 66 patients with Bell's palsy. According to the early response of BR(Rl), the patients were divided into 3 groups: Rl13ms(22cases), and absent BR(27cases). There was a remarkable tendency towards a satisfactory recovery from paralysis if BR occurred during the first 2 weeks from onset(p or = 25% (35cases), and CMAP amplitude comparison 3.5mA (23cases). When the threshold of electrical excitability on both sides differs 3.5mA or more an unsatisfactory recovery has strongly to expected(p<0.001). In patients with less than 3.5mA of NET threshold, 81.4% had a satisfactory recovery, but in patients with more than 3.5mA of NET threshold, 60.9% had a bad prognosis. BR, side-to-side CMAP amplitude comparison, and side-to-side NET threshold difference seem to be useful independent indices for predicting the prognosis an early stage of the paralysis.

A case Report of a Spontaneous cervical Hematomyelia

The spontaneous hematomyelia is an uncommon event and its predisposing conditions are vascular malformation, syringomyelia, pregnancy and delivery, angioma, hemophilia, anticoagulant therapy, etc. We have recently experienced the patient with spontaneous onset and resolving hematomyelia in the cervical spinal cord. A 30-year-old male patient with non-traumatic spinal shock was evaluated. On MRI, a hematomyelia along cervical spi-nal cord was revealed. A suspicious AV malformation was noticed at C3-4 level. Fol-low-up MRIs showed spontaneous resolution of the hematoma.

A Case of Hereditary Spastic Ataxia

Hereditary spastic ataxia is a familial neurological disorder which exhibit the features of a progressive combined pyramidal tract and cerebellar deficiency. The main features are progressive gait disturbance, incoordination, nystagmus, visual impairment, hyperreflexia, extensor plantar response, peripheral neuropathy, and pes cavus. A 27-year-old male patient with spastic ataxic gait was evaluated. He showed characteristic features of hereditary spastic ataxia. There were another twelve affected members in four generations of his family which may be inherited by autosomal dominant pattern. One of them is reported with review of the literature on familial spastic ataxia.

A Study of Pattern Reversal Visual Evoked Potential and Flash Electroretinogram in Patients with Optic Atrophy

The pattern reversal visual evoked potential(PRVEP) and flash electroretinogram(flash ERG) were performed in 22 patients with optic atrophy. Patients with ophthalmologic problems other than optic atrophy or with systemic disorders were excluded from the analysis The results are as follows: 1. In the 41 eyes of patients with optic atrophy, 39 of them showed abnormal PRVEP, in which all the eyes had no consistent waveform except in one patient 2 eyes with delayed P1 latency. 2. 13 eyes were abnormal in both PRVEP and flash ERG but no eye was abnomnal in flash ERG only 3, Regarding the flash ERG examination, 13 eyes were abnomlal. Of these, there was a period of 1 to 2 years for 1 eye's disease, a period of 2 to 5 years for another eye' disease and after 5 years 11 eyes were diseased. Therefore, it showed that the longer the duration of disease lasted, the more flash ERG abnormalities developed. 4. The abnormalities of PRVEP haxe no significant relationship with the duration of the disease.