ABSTRACT
OBJECTIVE: The purpose of this study is to evaluate the effect of the corpus callosotomy and to elucidate possible prognostic factors. METHODS: The cases of 39 patients who underwent corpus callosotomy were reviewed retrospectively. Clinical outcomes were analyzed using Engel's classification, with consideration of various presurgical conditions and the extent of the callosal resection during follow-up more than one year. RESULTS: Satisfactory outcome (Engel's class I, II) was obtained in 20 patients (51%) of 39 patients. In 36 cases with drop attack seizures, the class I, II outcomes were 22 patients (61%). When the patients were grouped according to the extent of callosal resection, the class I, II outcomes were 50% of the patients with anterior 1/2 or 2/3, 50% of those with anterior 4/5 callosotomy, and 57% of those with total callosotomy, respectively. The mean follow-up period was 34 months (24 to 58 months). CONCLUSION: Although it is not statistically significant, the patients who had underwent total callosotomy show better outcomes than those with partial callosotomy. Corpus callosotomy is efficacious in controlling medically intractable epilepsy in appropriately selected patients.
Subject(s)
Humans , Classification , Epilepsy , Follow-Up Studies , Retrospective Studies , Seizures , SyncopeABSTRACT
We report a case of acute transient encephalopathy with mental alteration, myoclonic jerks, and periodic triphasic wave electroencephalographic patterns caused by a therapeutic dose of baclofen. The clinical and electroencephalo-graphic abnormalities improved to a normal range shortly after baclofen was discontinued. We discuss the pathogenesis and review the literature about baclofen-induced encephalopathies.
Subject(s)
Baclofen , Myoclonus , Reference ValuesABSTRACT
Dilated cardiomyopaty is a rare occurrence in Hecker muscular dystropy. We report a case of Becken muscular dystrophy in 28 old man who presented with dyspnea, progressive muscle weakness, and dilated cardiomyopathy with severe left ventricular dysfunction which was detected by echocardiograpy. Muscle biopsy demonstrated diffuse degenerated changes consistent with progressive muscular dystropy, His brother has also Becker muscular dystrophy and dilated cardiomyopathy, The patient is on NYHA Class II~III with medical therapy.
Subject(s)
Humans , Biopsy , Cardiomyopathy, Dilated , Dyspnea , Muscle Weakness , Muscular Dystrophies , Muscular Dystrophy, Duchenne , Siblings , Ventricular Dysfunction, LeftABSTRACT
In 17 myotonic cases (16 of myotonic muscular dystrophy and 1 of congenital myotonia), 13 cases showed significant decremental responses on RNS study. The decremental responses had the tendency of more prominence on faster stimulating rate, and in the proximal limb muscles. This result suggests that the RNS test is useful but not specific for the disorder of neuromuscular junction.
Subject(s)
Humans , Extremities , Muscles , Muscular Dystrophies , Neuromuscular JunctionABSTRACT
There are widely recognized unusual remote neurological manifestations of internal malignancy. However, as far as the author's knowledge, the generalized myokymia associated with myokymic discharge has not been reported as paraneoplastic syndrome earlier. The patient was a 63-year-old female with generalized myokymia and paresthesia for 4months. The nerve conduction study revealed mild sensorimotor polyneuropathy and EMG study showed the generalized myokymic discharges, which were not changed by sleeping or brachial plexus block. The carbamazepine reduced the amount of myokymia and myokymic discharges but not the benzodiazepine. The diagnosis of lung cancer was confirmed by needle aspiration biopsy, and the cell types of lung cancer were mixed undifferentiated and small cell type. By the administration of anti-cancer drugs including cisplatin, the myokymia and myokymic discharges were completely disappeared. The patient died at 8 months after the diagnosis.
Subject(s)
Female , Humans , Middle Aged , Benzodiazepines , Biopsy, Needle , Brachial Plexus , Carbamazepine , Cisplatin , Diagnosis , Lung Neoplasms , Lung , Myokymia , Needles , Neural Conduction , Neurologic Manifestations , Paraneoplastic Syndromes , Paresthesia , PolyneuropathiesABSTRACT
Repetitive nerve stimulation test (RNS) is an easy and non-invasive test which provides objective for the presence of a myasthenic neuromuscular defect and for monitoring possible improvement through various therapeutic measures, and makes the differentiation of neuromuscular junction disorders. Analysing the RNS test of 45 normal controls and 146 patients with myasthenia gravis quantitatively, the following results obtained. 1. At low rate stimulation, the decremental response upto 2 standard deviation in normal control are 7.1% in orbicularis oculi(~), 7.6% in flexor carpi ulnaris (FCU) and 5.4% in abductor digiti guinti muscle(ADQ). There are two kinds of facilitation noticed: incremental responses at repetitive low stimulation (13.3-14.9%) immediately after tetanic stimulationy and increased mean amplitudes of compound muscle action potentials after exercise(l3-17%). Four minutes after the tetanic stimulation, the decremental responses at low rate stimulation become accentuated slightly even in normal control group. 2. At low rate stimulation. There are singificant decremental decremental responses in patients with myasthenia gravis, but statistically no significant differences are seen in the quantity of decremental responses among 2, 3 and 5/sec rate of stimulation. 3. The pattern and severity of decremental responses at low rate stimulation are depending on the clinical type of generalized myasthenia gravis. There are no decremental responses in FCU or ADQ in ocular type. The decremental responses of oo are greater than that of FCU in mild generalize myasthenia, but the reverse is true in moderate generalized type. 4. Statistically significant post-tetanic facilitation and exhaustion are noticed in patients with generalized myasthenia gravis.
Subject(s)
Humans , Action Potentials , Myasthenia Gravis , Neuromuscular Junction DiseasesABSTRACT
Repetitive nerve stimulation test (RNS) is an easy and non-invasive test which provides objective for the presence of a myasthenic neuromuscular defect and for monitoring possible improvement through various therapeutic measures, and makes the differentiation of neuromuscular junction disorders. Analysing the RNS test of 45 normal controls and 146 patients with myasthenia gravis quantitatively, the following results obtained. 1. At low rate stimulation, the decremental response upto 2 standard deviation in normal control are 7.1% in orbicularis oculi(~), 7.6% in flexor carpi ulnaris (FCU) and 5.4% in abductor digiti guinti muscle(ADQ). There are two kinds of facilitation noticed: incremental responses at repetitive low stimulation (13.3-14.9%) immediately after tetanic stimulationy and increased mean amplitudes of compound muscle action potentials after exercise(l3-17%). Four minutes after the tetanic stimulation, the decremental responses at low rate stimulation become accentuated slightly even in normal control group. 2. At low rate stimulation. There are singificant decremental decremental responses in patients with myasthenia gravis, but statistically no significant differences are seen in the quantity of decremental responses among 2, 3 and 5/sec rate of stimulation. 3. The pattern and severity of decremental responses at low rate stimulation are depending on the clinical type of generalized myasthenia gravis. There are no decremental responses in FCU or ADQ in ocular type. The decremental responses of oo are greater than that of FCU in mild generalize myasthenia, but the reverse is true in moderate generalized type. 4. Statistically significant post-tetanic facilitation and exhaustion are noticed in patients with generalized myasthenia gravis.
Subject(s)
Humans , Action Potentials , Myasthenia Gravis , Neuromuscular Junction DiseasesABSTRACT
Sulpiride, a selective antagonist for adenylate cyclase-independent dopamine receptors, was administrated to 25 patients with blepharospasm and oromandibular dystonia(Meige's syndrome). Of the 25, 7 patients (28%) exhibited marked and lasting improvement with sulpiride and 12 patients (48%) showed mild or transient improvement. This favorable therapeutic response to sulpiride suggests that striatal glutamate underactivity may play a role in the pathophysiology of Meige's syndrome as a primary or secondary defect.
Subject(s)
Adult , Aged , Female , Humans , Male , Basal Ganglia Diseases/drug therapy , Corpus Striatum/metabolism , Dopamine/metabolism , Glutamates/metabolism , Glutamic Acid , Meige Syndrome/drug therapy , Middle Aged , Sulpiride/therapeutic useABSTRACT
One month following carbon monoxide poisoning, a 39 year-old man developed incontinence, memory impairment, disorientation and emotional instability. He was hospitalized 7weeks later, and during hospitalization he exhibited myoclonic movements of the neck and lower limbs. He was given piracetam intravenously for 11 days. The myoclonus was significantly reduced by the third day of treatment and had disappeared by the seventh day. There was no recurrence following cessation of treatment.
Subject(s)
Adult , Humans , Male , Carbon Monoxide Poisoning/complications , Myoclonus/drug therapy , Myoclonus/etiology , Piracetam/therapeutic use , Pyrrolidinones/therapeutic useABSTRACT
There has been considerable controversy concerning divergence paralysis, an entity described as early as 1883 by Parinaud. We recently observed a patient with divergence paralysis, who on CT scan presented a small hematoma in the tegmentum of the brainstem. This case may support the theory that the center for divergence exists in the upper brainstem.
Subject(s)
Humans , Male , Cerebral Hemorrhage/complications , Cerebral Hemorrhage/diagnostic imaging , Hematoma/complications , Hematoma/diagnostic imaging , Middle Aged , Paralysis/etiology , Tegmentum Mesencephali/diagnostic imaging , Tomography, X-Ray ComputedABSTRACT
Observing a 16 years old male of classical Friedreich's ataxia with progressive ataxia, dysarthria, areflexia, loss of vibration and proprioception, Babinski sign, pes cavus, scoliosis, hyperglycemia, optic atrophy and hearing deficits, the nerve conduction studies and several evoked potential studies are performed. The sensory nerve potentials are markedly decreased in amplitude and the average technique has to be used to get the potentials, worse distally. The motor nerve functions, however, are normal. There is no consistent waves recorded on BAEP even with maximal stimulation bilaterally. VEP reveals prolonged latencies bilaterally but with normal amplitudes. On median nerve SEP stimulated at wrist, the amplitudes of potentials at Erb's point look decreased and no definite evoked potentials are recored at contralateral scalp. In contrast, the cervical evoked potentials are normal in amplitudes and latencies.