Measurement of motor root conduction time at the early stage of Guillain-Barre syndrome.

BACKGROUND AND PURPOSE: As they are mainly performed at distal nerve parts, routine electrophysiological examinations can fail to detect the abnormalities at the early stage of Guillain-Barre syndrome (GBS) because of predominant involvement of proximal nerve segments. Measurements focused on proximal conduction can provide additional findings. We investigated the diagnostic significance of motor root conduction time (MRCT) at the early stage of GBS. METHODS: Study was performed in 30 healthy volunteers and within the first week of 12 patients with GBS. MRCT was calculated as the latency differences between motor nerve conduction time (MNCT) obtained by cervical magnetic stimulation and total peripheral motor conduction time obtained by electrical stimulation of ulnar nerve. Also MNCT/MRCT ratio was calculated in each subject. RESULTS: There were statistically significant differences between groups for MRCT (P < 0.0001) and MNCT/MRCT ratio (P < 0.0001). Although the F-wave latency at ulnar nerve was abnormal only in 33%, MRCT was significantly prolonged in 83% of patients. In three patients, prolongation of MRCT was the only abnormality at the first electrodiagnostic examination. CONCLUSIONS: Motor root conduction time as a non-invasive method can provide additional and confirmative information at the early stage of GBS in which routine nerve conduction studies may fail to detect the focal demyelination.

Physiotherapy approaches in the treatment of ataxic multiple sclerosis: a pilot study.

OBJECTIVE: This study was planned to investigate the efficacy of neuromuscular rehabilitation and Johnstone Pressure Splints in the patients who had ataxic multiple sclerosis. METHODS: Twenty-six outpatients with multiple sclerosis were the subjects of the study. The control group (n = 13) was given neuromuscular rehabilitation, whereas the study group (n = 13) was treated with Johnstone Pressure Splints in addition. RESULTS: In pre- and posttreatment data, significant differences were found in sensation, anterior balance, gait parameters, and Expanded Disability Status Scale (p < 0.05). An important difference was observed in walking-on-two-lines data within the groups (p < 0.05). There also was a statistically significant difference in pendular movements and dysdiadakokinesia (p < 0.05). When the posttreatment values were compared, there was no significant difference between sensation, anterior balance, gait parameters, equilibrium and nonequilibrium coordination tests, Expanded Disability Status Scale, cortical onset latency, and central conduction time of somatosensory evoked potentials and motor evoked potentials (p > 0.05). Comparison of values revealed an important difference in cortical onset-P37 peak amplitude of somatosensory evoked potentials (right limbs) in favor of the study group (p < 0.05). CONCLUSIONS: According to our study, it was determined that physiotherapy approaches were effective to decrease the ataxia. We conclude that the combination of suitable physiotherapy techniques is effective multiple sclerosis rehabilitation.

A new model for tethered cord syndrome: a biochemical, electrophysiological, and electron microscopic study.

In order to investigate the pathophysiology of the tethered cord syndrome, a few experimental models have been developed and used previously. In this study, the authors present a new experimental model to investigate the biochemical, electrophysiological, and histopathological changes in the tethered spinal cord syndrome. A model was produced in guinea pigs using an application of cyanoacrylate to fixate the filum terminale and the surrounding tissue to the dorsal aspect of the sacrum following 5-gram stretching of the spinal cord. The experiments were performed on 40 animals divided into two groups. The responses to tethering were evaluated with hypoxanthine and lipid peroxidation, somatosensory and motor evoked potentials, and transmission electron microscope examination. The hypoxanthine and lipid peroxidation levels significantly increased, indicating an ischemic injury (p < 0.01). The average hypoxanthine level in the control group was 478.8 +/- 68.8 nmol/g wet tissue, while it was 651.2 +/- 71.5 nmol/g in the tethered cord group. The lipid peroxidation level in group I was 64.0 +/- 5.7 nmol/g wet tissue, whereas it was 84.0 +/- 4.7 nmol/g in group II. In the tethered cord group, the latencies of the somatosensory and motor evoked potentials significantly increased, and the amplitudes decreased. These changes indicated a defective conduction in the motor and sensorial nerve fibers. In the transmission electron microscopic examinations, besides the reversible changes like edema and destruction in the gray-white matter junction, irreversible changes like scarcity of neurofilaments and destruction in axons and damage in myelin sheaths were observed. We consider that this work can be used as an experimental model for tethered cord syndrome.

PCR detected hepatitis C virus genome in the brain of a case with progressive encephalomyelitis with rigidity.

A case of progressive encephalomyelitis with rigidity (PEWR) associated with hepatitis C virus (HCV) is reported. A 58 year-old woman presented with a clinical picture of progressive quadriparesis, sensory loss, sphincter dysfunction, painful muscle spasms in the upper and lower limbs and continuous muscle unit activity in electromyography. She developed hepatitis, pancreatitis and HCV-RNA was detected in the plasma by reverse transcription-polymerase chain reaction (RT-PCR). Postmortem histopathological examination showed encephalomyelitis with perivascular lymphocyte cuffing, infiltration and neuronal loss mainly affecting the brainstem and cervical spinal cord. The RT-PCR analysis of the postmortem brain, brainstem, liver, pancreas, plasma and CSF samples revealed the presence of HCV genome in all specimens except CSF. Clinical features, postmortem histopathology and PCR results and the possible etiopathogenesis of PEWR are briefly discussed.

Efficacy of high dose amino acid solution on spinal cord injury induced by focal Nd:YAG laser irradiation.

In this experimental study, a neodymium:yttrium-aluminium-garnet (Nd:YAG) laser was used to induce highly reproducible focal spinal cord lesions in anaesthetized guinea pigs. The efficacy of high dose amino acid solution (HDAAS) on this injury is investigated. Experiments were performed on 36 animals divided into three groups; sham operated controls, laser irradiated surgical controls, and amino acid groups. Acute responses to injury were evaluated with somatosensory (SSEP) and motor evoked potentials (MEP) and functional recovery was assessed for 8 weeks using the inclined plane technique. In the laser irradiated surgical control group, MEP disappeared one hour after the laser injury, but SSEP revealed changes of amplitude and latency. In this group, the average value of the inclined plane at 24 hours after the laser application was 45.3 +/- 1.4 degrees. In the amino acid group, at the sixth hour of injury, MEP and SSEP changes improved with infusion of HDAAS for 4 hours. This improvement was statistically significant (for latency of SSEP U = 140 p < 0.05). Inclined plane value at 24 hours after the laser application was 65.5 +/- 1.2 degrees in this group. This study showed that application of Nd:YAG laser irradiation on the spinal cord induced spinal cord injury which presented as paraparesis, HDAAS may provide significant therapeutic protection in secondary damage following this injury and may have a potential role in the treatment of acute spinal cord injury.

A new experimental model: for further investigation of the secondary changes following spinal cord injury.

To investigate the pathophysiology of spinal trauma, several experimental models have been used. In this study, the authors present a new experimental model for further investigation of secondary damage following spinal injury. Nd:YAG laser irradiation was applied to the spinal cord of guinea pigs to produce an injury. A 30 Watt (20 Joule) laser produced paraparesis. The injury was evaluated with evoked potentials, lipid peroxidation values, and histopathological examination.

Brainstem auditory evoked potentials and blink reflexes in patients with pontocerebellar angle tumors.

Brainstem auditory evoked potentials (BAEP) and blink reflexes (BR) were obtained from 13 patients with pontocerebellar angle (PCA) tumors whose pathological diagnosis was as follows: 7 acoustic neurinoma, 3 meningioma, 1 neurinoma, 1 brain stem epidermoid tumor, 1 arteriovenous malformation. The most prominent abnormality was noted in BAEP generated by stimulating the ear ipsilateral to the lesion and ipsilateral R1 reflexes obtained from ipsilateral stimulation of the supraorbital nerve.

Late visual recovery after intravenous methylprednisolone treatment of Purtscher's retinopathy.

A 25-year-old man involved in a minor traffic accident subsequently had a Purtscher's-type retinopathy and lost visual acuity (20/800). After treatment with intravenous methylprednisolone at a dose of 1 g/day, his visual acuity improved to 20/70 three days later and to 20/50 one week later. The exudates and hemorrhages gradually disappeared. However, a localized central scotoma and afferent pupillary defect still persisted. We review previous reports on the retinal pathophysiology of Purtscher retinopathy and discuss the potential benefit of treatment with high-dose intravenous corticosteroids.

Evoked potentials in Guillain-Barré syndrome.

A prospective evaluation was made in a total of 19 patients with Guillain-Barré syndrome (GBS) by neurologic examination, visual evoked potentials (VEP), brain-stem auditory evoked potentials (BAEP) and somatosensory evoked potentials (SEP) during the acute phase of the disease, three weeks later, and then again three months after the onset of symptoms. The results of electrophysiologic studies of the patients were compared with those of 19 healthy children. There were no statistically significant differences found in the mean VEP and BAEP values between the patient and control groups. However, the VEP and BAEP values were abnormal during the first and second stages of the disease in only a few patients. There was a statistically significant prolongation of the response in tibial and median SEP during the first and second stages of the disease when compared with the third stage and the control group. The Erb-Cervical 7 (C7) interwave latency was elevated in the median SEP. It was concluded that the prolongation of the response in SEP was diagnostically important in the acute phase of GBS. There was no correlation found between SEP and clinical progression.

Neurophysiological studies of patients with classical phenylketonuria: evaluation of results of IQ scores, EEG and evoked potentials.

Neurophysiological studies were conducted in 42 patients with classical phenylketonuria. The results of the intelligence quotient scores, electroencephalogram, visual evoked potentials and brain-stem auditory evoked potentials were evaluated. When compared with the controls, the subjects demonstrated a significant prolongation in VEP P1 and BAEP I-V interpeak latencies and an increase in VEP N1P1 amplitudes. No relationship was found between these pathological responses and metabolic control. However, the observation of normal intelligence quotient scores in 14 out of 18 patients who displayed a pathological prolongation in P1 latencies led us to the conclusion that evoked potentials may have a significant role in the determination of neurophysiological defects and that even cases with good metabolic control may have some obscure neurophysiological dysfunction which should be evaluated more carefully.

Occidental type cerebromuscular dystrophy: a report of eleven cases.

Occidental type cerebromuscular dystrophy (OCMD) forms a substantial distinct group within congenital muscular dystrophy (CMD). These patients invariably present with amyotrophy, multiple joint contractures, facial muscle involvement, normal or nearly normal intelligence, leukodystrophic appearance on CT scan, and dystrophic changes in muscle.

Fukuyama type congenital muscular dystrophy in a Turkish child.

Congenital muscular dystrophy (CMD) is a heterogeneous group of disorders which is associated with more or less degrees of cerebral involvement. There are four separate entities within CMD nosology. Among these Fukuyama's CMD (FCMD) is highly prevalent in Japan, whereas the classic form with normal or subnormal intelligence, also known as the occidental type, covers the vast majority of cases in the West. We report a case of FCMD seen in a Turkish child.