Miller-Fisher syndrome (Guillain-Barré syndrome with ophthalmoplegia) during treatment with gold salts in a patient with rheumatoid arthritis.
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Biomedical subjects
Publications and source records attributed to J Carbonell.
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The effects of gibberellic acid (GA(3)) and calcium ions on the production of alpha-amylase and acid phosphatase by isolated aleurone layers of barley (Hordeum vulgare L. cv Himalaya) were studied. Aleurone layers not previously exposed to GA(3) or Ca(2+) show qualitative and quantitative changes in hydrolase production following incubation in either GA(3) or Ca(2+) or both. Incubation in H(2)O or Ca(2+) results in the production of low levels of alpha-amylase or acid phosphatase. The addition of GA(3) to the incubation medium causes a 10- to 20-fold increase in the amounts of these enzymes released from the tissue, and addition of Ca(2+) at 10 millimolar causes a further 8- to 9-fold increase in alpha-amylase release and a 75% increase in phosphatase release. Production of alpha-amylase isoenzymes is also modified by the levels of GA(3) and Ca(2+) in the incubation medium. alpha-Amylase 2 is produced under all conditions of incubation, while alpha-amylase 1 appears only when layers are incubated in GA(3) or GA(3) plus Ca(2+). The synthesis of alpha-amylases 3 and 4 requires the presence of both GA(3) and Ca(2+) in the incubation medium. Laurell rocket immuno-electrophoresis shows that two distinct groups of alpha-amylase antigens are present in incubation media of aleurone layers incubated with both GA(3) and Ca(2+), while only one group of antigens is found in media of layers incubated in GA(3) alone. Strontium ions can be substituted for Ca(2+) in increasing hydrolase production, although higher concentrations of Sr(2+) are required for maximal response. We conclude that GA(3) is required for the production of alpha-amylase 1 and that both GA(3) and either Ca(2+) or Sr(2+) are required for the production of isoenzymes 3 and 4 of barley aleurone alpha-amylase.
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The authors report the electroclinical data in a left-handed patient presenting with a partial paroxystic state immediately following cranial trauma complicated by a right temporal subdural hematoma. The clinical picture during the crisis was limited to symptoms evoking a subjective post-traumatic syndrome. Anti-convulsant treatment produced complete disappearance of clinical disorders and critical focal activity. The problems raised by this observation are discussed.
Twenty-three schizophrenics, ten psychiatric controls, and 17 normal controls were used to test the hypothesis that schizophrenics suffer a deficit in their ability to integrate information from different sensory modes. The task involved identifying auditory, visual, or mixed (auditory and visual) patterns which had previously been equated in difficulty for normal subjects. Mean error scores were greatest for schizophrenics and least for normals with psychiatric controls in between. Moreover, the schizophrenics did equally well whether the task was visual, auditory, or mixed. Thus, schizophrenics showed no deficit specific to the synthesis of information from two different sensory modes.
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A 24-year-old man survived for 8 years after a vascular lesion of the pons and midbrain. During these years a subresponsive comatose state with neurological signs extending from the oculomotor nuclei to the trigeminal and facial nuclei levels was present. A decorticated type of rigidity, with bilateral paralysis of the limbs accompanied by pyramidal signs, was also present. The EEG during the first months showed slow activity which afterwards gradually changed towards fast and alpha activities, maintained in the course of the years. The pathological diagnosis was a fibrous endarteritis, mainly in the territory of the basilar and vertebral arteries. The pons and midbrain, presenting a large cystic infarct, were serially studied to determine the anatomical extent of the lesion. After reviewing other examples from the literature, the clinical features of our case are discussed. The evolution of the EEG is related to different structures of the ponto-mesencephalic region with different functional activities.
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Acral lentiginous melanoma is a very aggressive, malignant cutaneous tumor which has a predilection for the plantar surface of the feet, palms of the hand, and the digits. The lesion presents as a rapidly spreading, darkly pigmented patch and may display varying degrees of pigmentation. These lesions have a propensity to metastasize to the central nervous system, liver, lungs, bone, and lymph nodes. Due to the aggressive nature of these lesions, early recognition and treatment, consisting of excision in toto with clean margins, are imperative. The authors present a thorough review of the literature and an illustrative case of acral lentiginous melanoma of the lower extremity.
INTRODUCTION: Recently a series of cases has been reported characterized by myoclonic crises similar to those occurring in benign myoclonic epilepsy of childhood. However, these crises only occurred after unexpected tactile or auditory stimuli. These clinical conditions represent a new epileptic syndrome, which is age-dependent and has been called benign myoclonic epilepsy of childhood. CLINICAL CASE: We present the case of a 12 month old girl with myoclonic crises which occurred only after auditory or tactile stimuli. The myoclonia could be set off whilst awake or asleep. No other types of crises or neurological changes were seen. A brother of the patient had had febrile convulsions. The EEG recorded during the crises showed generalized brief spike-and-wave discharges at 3 cycles/second. The intercritical EEG was normal whilst awake, but during sleep showed brief generalized discharges. After treatment with valproate was started the crises became less frequent. CONCLUSIONS: The case we describe is similar to those described by Ricci et al in 1995. We, therefore, consider it to fit the concept of reflex myoclonic epilepsy of childhood of benign character. We consider that this condition should be differentiated from other reflex epilepsies and epileptic syndromes with a predominance of myoclonia, including benign myoclonic epilepsy of childhood.
INTRODUCTION AND OBJECTIVE: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system. This study is based on clinical symptoms and diagnostic tests employed. PATIENTS AND METHODS: We describe a seven children series indicating the initial neurologic abnormalities, diagnostic tests, treatments used and clinical-neuroradiological evolution. RESULTS: The mean presentation age was 4.1 years. Initial neurologic symptoms were mainly spastic hemi/paraparesis, cerebellous and pyramidal syndrome, consciousness changes, meningeal signs, seizures and cranial nerve palsies. The cerebrospinal fluid was abnormal in four patients with positive serologic tests in two of them (Coxsackie B). Electrophysiological studies were affected in 50%. MRI findings consisted of multifocal supratentorial white matter lesions. Clinical evolution revealed a progressive improvement with resolution after two months. Follow-up was made between six months and five years. The treatment was based on aciclovir and corticosteroids. CONCLUSIONS: ADEM runs a monophasic course of progressive neurologic abnormalities. Diagnosis is based on suggestive clinical and neuroimaging findings. Generally speaking, MRI showed resolution of multifocal lesions in conjunction with clinical improvement.
INTRODUCTION: Acquired oculomotor palsy in juvenile age are most commonly due to head trauma, tumors, migraine, vasculopathies and demyelinating diseases. CLINICAL CASES: We document three cases of paroxysmal oculomotor nerve palsy in childhood, illustrating the main clinical symptoms, neurological examination, MR images, diagnosis, treatment and evolution. Final diagnosis were: multiple sclerosis, pineoblastoma, and craniopharyngioma. CONCLUSIONS: Sudden oculomotor nerve palsy occurring in youth in the absence of head trauma, viral infection or migraine access, firmly suggests a serious intracranial disease, and neuroimage studies should be quickly obtained to have an early diagnosis.