A Roles of Cinedefecography and Electromyography in the Diagnosis of Paradoxical Puborectalis Syndrome

BACKGROUND: Paradoxical puborectalis syndrome (PPS) is a complex and poorly understood entity characterized by contraction rather than relaxation of the pelvic floor muscles during attempted evacuation. Anal electromyography (EMG) and cinedefecography (CD) are the most commonly used tests for the diagnosis of PPS. Therefore, the aim of this study was to prospectively assess the correlation of EMG and CD in the diagnosis of PPS. METHODS: All patients with symptoms of obstructed evacuation who underwent EMG and CD between September 1998 and February 1999, were evaluated. The clinical criteria for PPS included incomplete or difficult evacuation, straining, tenesmus, and the need for an enema or digitation. The EMG criteria included failure to achieve a significant decrease in the electrical activity of the puborectalis during attempted evacuation. The CD criteria included either paradoxical contraction or failure of the puborectalis to relax along with incomplete evacuation. RESULTS: Twenty-nine (29) patients had clinical evidence of PPS, 8 males and 21 females with a mean age of 42.6 years (range, 19-75 years), and underwent CD and EMG. The mean duration of symptoms was 7.2 years (range, 3 months-30 years). Nineteen patients (65.5%) had evidence as having PPS on CD and/or EMG. Of these patients, six patients (20.7%) were diagnosed as having PPS on both tests, five patients (17.2%) were only diagnosed on CD, and eight patients (27.6%) were only diagnosed on EMG. The remaining ten patients had normal puborectalis muscle relaxation on CD and EMG. Therefore, the correlation rate between the two tests was 55.2%. If EMG was considered as the ideal test for the diagnosis of PPS, CD had a sensitivity of 42.9% and a specificity of 66.7%. Conversely, if CD was considered as the ideal test, EMG had a sensitivity of 54.5% and a specificity of 55.6%. CONCLUSIONS: The sensitivity and the specificity values of EMG and CD for the diagnosis of PPS are suboptimal, and a low correlation existed between the two tests. This result suggests that the diagnosis of PPS should not be based upon only one test.

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.