Cases reported "Consciousness Disorders"

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1/11. Type II (adult onset) citrullinaemia: clinical pictures and the therapeutic effect of liver transplantation.

    OBJECTIVE: adult onset type II citrullinemia is an inherited disorder of amino acid metabolism caused by a deficiency of liver specific argininosuccinate synthetase activity. Most of the patients with this disease were reported in japan and therefore, this disease has not been well recognised outside this country. The detailed clinical pictures of the patients with type II citrullinaemia are reported and their outcomes after liver transplantation referred to. methods: Ten patients with this disease were evaluated. Seven of them underwent liver transplants using a graft obtained from a healthy family member. RESULTS: There were six men and four women; the age of onset of encephalopathy ranged from 17 to 51 years. The initial symptom in nine patients was sudden onset disturbance of consciousness, and one patient had long been regarded as having a chronic progressive psychotic illness. High concentrations of plasma citrulline and ammonia were commonly seen on admission. Although brain CT or MRI lacked any consistent findings, the EEG was abnormal in all patients, showing diffuse slow waves. Additionally, in five patients chronic pancreatitis preceded the onset of encephalopathy. After liver transplantation the metabolic abnormalities, including abnormal plasma concentrations of citrulline and ammonia, were immediately corrected and all neuropsychic symptoms soon disappeared, except for impaired cognitive function in one patient. Six out of these seven patients returned to their previous social lives, including work. CONCLUSIONS: The clinical concept of adult onset type II citrullinaemia coincides well with the range of hepatic encephalopathy, and liver transplantation is a very promising therapeutic approach.
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2/11. Triphasic waves and spike wave stupor.

    Triphasic waves are usually thought of as indicating a metabolic encephalopathy. Recent investigations have added nonmetabolic etiologies to the differential diagnosis of triphasic waves. seizures are not generally thought of as associated with triphasic waves. Similarities in the appearance of records with encephalopathies and continuous triphasic waves and those of some patients with the Lennox-Gastaut syndrome have been noted. We presented a case which suggests that the presence of TW in a patient with a metabolic encephalopathy might suggest petit mal status epilepticus.
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3/11. Treatment of neurological complications in isolated ACTH deficiency by glucocorticoid replacement.

    The neurophysiological complications in ACTH deficiency have not been well documented. In this paper, we present a patient with isolated ACTH deficiency who developed various neurological signs. The neurophysiological abnormalities, including slow wave activity on electroencephalogram, delayed conduction velocity of the peripheral nerves and low amplitude of muscle action potentials, were improved by replacement of glucocorticoid. These findings suggested that glucocorticoid is directly involved in the function of the peripheral and central nervous systems.
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4/11. A case of epilepsy characterized by spike-wave stupor followed for long period.

    A 34-year-old housewife with nonconvulsive status epilepticus was followed up for 20 years since the initial fit. She maintained some contact with reality during the stupor and recalled the episode to some extent, while the EEG showed continuous spike-wave complexes. During the clinical course, the main type of seizures was spike-wave stupor, of which the maximum frequency was several times a week in the hospital and the duration was many minutes to several hours, and also secondarily generalized convulsive seizures occurred approximately once a month. The lasting control of spike-wave stupor was not achieved in spite of the various medications for the long follow-up period. The ictal EEGs of spike-wave stupor always showed the frontal origin. The meaning of the term "nonconvulsive status epilepticus" in this case was briefly discussed.
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5/11. A female case with the kleine-levin syndrome and its physiopathologic aspects.

    A female case with the kleine-levin syndrome (KLS), which first occurred at the age of 19, was discussed in relation to the following four characteristics: 1) a female case, 2) a loss of memory and the appearance of slow waves in the pathosis, 3) the abnormal pattern of growth hormone (GH) secretion during sleep in the pathosis, and 4) a prolonged latency between the peaks of III and V in the auditory brainstem response (ABR) in both the pathosis and nonpathosis. These characteristics may suggest that there is a slight disturbance of consciousness in the pathosis, and that there is a functional disturbance in the hypothalamus.
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6/11. Episodic twilight state with severe autonomic symptoms in an epileptic patient.

    A boy suffering from epileptic seizures experienced five episodes of a twilight state. His twilight states continued for at least two weeks, and were characterized by behavioral disorders and severe autonomic symptoms. Sometimes functional ileus was present. The electroencephalographic finding in the first episode was 6-per-second phantom spike and wave complex (PSW). For treatment, a combination of carbamazepine and sodium valproate was useful in preventing the reappearance of the episode of the twilight state and in suppressing PSW. From the clinical and electroencephalographic findings and therapeutic response to antiepileptics, the episodes were considered to have originated in localized epileptic discharges in the hippocampal, amygdaloidal and hypothalamic regions.
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7/11. Classic migraine attack complicated by confusional state: EEG and CT study.

    We report electroencephalographic and clinical observations in one attack of classic migraine, accompanied by a confusional state. The ictal EEG showed slow waves over the left hemisphere, the abnormality gradually clearing up during the four days following the attack. A brain CT scan was normal during the attack and also a few days later. A possible relationship between impairment of consciousness during the attack and left hemispheric metabolic dysfunction is discussed.
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8/11. Ictal clinical patterns and ictal EEG in a case of partial seizures of frontotemporal origin associated with complex symptomatology.

    The patient was a 35-year-old, unmarried male whose epileptic psychomotor fits persisted since the age of 13. The author has observed the case for about 12 years, so that incomplete information concerning epileptic symptoms was considered to be compensated considerably by longitudinal observation, including ictal seizure and ictal EEG's. In this patient seizure with impaired consciousness which correspond rhythmic slow waves of EEG tracing might be a nuclear sign; several kinds of automatism then might be considered as postictal phenomenon. The most important of all was tonic seizure of psychomotor epilepsy particularly in the face which was not seen in the petit mal epilepsy. With observation of ictal period as well as ictal EEG, differential identification of centrencephalic epilepsy and psychomotor epilepsy may not be totally impossible. The case also showed a typical productive psychotic episodes of Landolt, which could be treated favorably by 10 mg of intravenous haloperidol. This method, named as "pathologization" of Helmchen, was found by the present author as a useful treatmental means of choice.
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9/11. Stuporous episodes during treatment with sodium valproate: report of seven cases.

    Of 13 patients with complex partial seizures who experienced stuporous states during treatment with sodium valproate (VPA), 4 received VPA only, 4 VPA and phenobarbital (PB) and 5 VPA, PB, and a third anticonvulsant. Seven cases were described in detail. Side effects-stupor or confusion-appeared a few days after efficacious drug plasma levels were attained, persisted until therapy was readjusted, and disappeared 24 to 72 h after VPA withdrawal. Therapeutic trials established the role of VPA in the onset of stuporous states. The adverse effects of VPA were potentiated by the concomitant administration of other anticonvulsants. stupor was not due to VPA overdoses, and plasma concentration of the drugs were not correlated with the electroclinical signs. The EEG showed spike and wave discharges or continuous sharp theta and delta waves persisting during VPA treatment. The fact that all 13 stuporous, VPA-treated patients were subjected to partial seizures with complex symptomatology, and none were cases of generalized epilepsy, together with the observations that the disturbances of consciousness started with focal symptoms and EEG signs resembling those of spontaneously occurring partial seizures, suggest that VPA given alone or in association with other antiepileptics has a paradoxical epileptogenic effect in certain forms of epilepsy.
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10/11. Impairment of consciousness induced by valproate treatment following neurosurgical operation.

    Eight patients, who underwent neurosurgery for various supratentorial lesions presented unexplained impairment of consciousness during the first post-operative days. These states ranged from stupor to deep coma, and two patients had an emergency re-exploration. All these patients were receiving the usual dosage (1000 to 1500 mg per day) of valproic acid (VPA). The treatment had been initiated either several weeks or months previously in 4 cases, and was thus well tolerated, or the day before surgery in the 4 other cases. EEG recordings displayed diffuse abnormalities, delta waves and/or high voltage triphasic complexes, that led to the diagnosis of VPA intolerance and drug withdrawal. Then full clinical recovery and EEG clearing occurred within 1 to 5 days. VPA intolerances are wellknown but remain exceptional, about 1 case per 100.000. In our neurosurgical population, however, the rate was higher, approximately 2%. Wide areas of blood brain barrier destruction may contribute to the higher frequency of this easily reversible cause of post-operative stupor or coma.
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