[Progressive primary aphasia: detection of an uncommon dementia in primary care].
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Biomedical subjects
Publications and source records attributed to A Martínez-Salio.
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The authors describe a patient who presented with myoglobinuria after starting cerivastatin-gemfibrozil therapy. Muscle histochemistry revealed ragged-red fibers and cytochrome c oxidase negative (COX) fibers, and biochemistry showed a defect of COX activity. Immunoblot analysis showed a 60% reduction of COX I and COX II polypeptides. Cerivastatin myotoxicity might be related to a depletion of essential metabolites needed to anchor COX subunit I to mitochondrial membrane.
INTRODUCTION: We analyse the demand for neurologic care reasons for consultation and diagnoses of the immigrant population in two extrahospitalary neurological outpatient clinics. PATIENTS AND METHODS: A prospective one-year study of first visits of adult immigrant patients sent to the neurological outpatient clinic. RESULTS: A total of 342 patients were included in the study. They represented 9.4% of total patients in one clinic and 4.1% in another. The average age was 32.9 years old. There was a predominance of women (62.2%). The average stay in our country was two years. Latin America (especially from Ecuador and Colombia), Morocco and East Europe were mainly the origin of the patients. The cause of consultation was headache in a half of the cases and neuromuscular pathology, epilepsy and psychiatric disorders were the next most frequent diagnoses in descending order. The most frequent diagnoses was tension-type headache, and in descending order of frequency: migraine and transformed migraine, epilepsy, carpal tunnel syndrome, idiopathic facial palsy, low back pain and sciatica and syncope. Tension-type headache and transformed migraine were in relation to their situation of immigrants (family, working, adaptive problems). CONCLUSIONS: The neurologic care demands in immigrant population is the same as in spanish one. These are a young people that fundamentally consults by headache (in relation to adaptive problems), neuromuscular disorders, epilepsy and psychiatric disorders. The public health service have to take into account this population to health resources planning.
OBJECTIVES: Some previous reports suggested a potential role of insulin in memory and in the pathophysiology of Alzheimer's disease (AD). We assessed the cerebrospinal fluid (CSF) levels of insulin in patients with AD and in age and sex-matched controls trying to elucidate whether this value could be related with the risk or severity of AD. PATIENTS AND METHODS: We measured the CSF insulin levels in 27 patients with AD and 16 matched controls using a RadioImmunoanalysis method. RESULTS: CSF insulin levels did not differ significantly between AD-patient and control groups. These values were not correlated with age, age at onset, duration of the disease, and scores of the MiniMental State Examination in the AD group. CONCLUSION: These results suggest that CSF insulin concentrations are not related with the risk or severity of AD.
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Reversible posterior leukoencephalopathy syndrome is a newly characterised and increasingly recognized clinico-radiologic syndrome. Underlying conditions that reportedly trigger this syndrome include hypertensive encephalopathy, eclampsia, renal failure, and immunosuppressive drug therapy with cyclosporine, tacrolimus and interferon alpha. We describe a 51-year-old woman with non-Hodgkin's lymphoma treated with conventional CHOP chemotherapy. Eight days after this treatment she developed severe headache, bilateral visual loss and focal seizures with secondary generalization. Neurologic examination showed confusion, cortical blindness, and left hemiparesis with hyperreflexia and sensory loss. A cranial T2-weighted magnetic resonance imaging revealed increased signal intensity in the occipital and frontal lobes in both hemispheres and right parietal lobe. A diagnosis of reversible posterior leukoencephalopathy was made. She presented a favourable outcome with conservative treatment with mannitol and phenytoin. A new cranial scanning showed nearly complete resolution of the abnormalities. To the best of our knowledge, this is the first case of reversible posterior leukoencephalopathy in a patient treated with standard-dose CHOP. In this patient, we confirm the theoretical pathophysiologic mechanisms suggested explaining how these drugs can cause the syndrome.
We compared serum levels of coenzyme Q10 and the coenzyme Q10/cholesterol ratio in 33 patients with Parkinson's disease (PD) and 31 matched controls. The mean serum coenzyme Q10 levels did not differ significantly between the 2 study groups. Coenzyme Q10 levels were not correlated with age, age at onset, duration of the disease, scores of the Unified Parkinson Disease Rating Scale (UPDRS) or the Hoehn and Yahr staging in the PD group. The coenzyme Q10/cholesterol ratio had a significant correlation (although low) with duration of the disease (r = -0.46), total UPDRS score (r = -0.39), motor examination of the UPDRS (r = 0.45). These values were not influenced significantly by therapy with levodopa or dopamine agonists. The normality of serum coenzyme Q10 and coenzyme Q10/cholesterol ratio suggest that these values are not related with the risk for PD.
To elucidate whether serum coenzyme Q10 levels are related with the risk for amyotrophic lateral sclerosis (ALS), we compared serum levels of coenzyme Q10 and the coenzyme Q10/cholesterol ratio, in 30 patients with ALS and 42 matched controls using a high performance liquid chromatography technique. The mean serum coenzyme Q10 levels and the coenzyme Q10/cholesterol ratio did not differ significantly between the 2 study groups. These values were not influenced by the clinical form (spinal vs. bulbar) of ALS, and they did not correlate with age, age at onset, and duration of the disease. These results suggest that serum coenzyme Q10 concentrations are unrelated with the risk for ALS.
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The coexistence of chronic paroxysmal hemicrania and trigeminal neuralgia is called chronic paroxysmal hemicrania-tic syndrome. We describe the case of a man who has suffered both types of pain occurring synchronously but with different localization on the ipsilateral side. The pain attacks could be abolished with indomethacin and carbamazepine. To the best of our knowledge, this is the third case to be reported, the first in the male sex. We review this new disorder and discuss the pathophysiology.
It has been suggested that nitric oxide (NO) could be implicated in the pathogenesis of multiple sclerosis (MS). Recently, two groups reported increased cerebrospinal fluid (CSF) nitrate levels (oxidation product that provides an indirect estimation of NO) in MS patients. However, another group did not confirm these findings. We studied the CSF and plasma levels of nitrate with a kinetic cadmium reduction method in 11 MS patients and 25 matched controls. The CSF nitrate levels and the CSF/plasma nitrate ratio did not differ significantly between the two study groups. Plasma nitrate levels were nearly significantly lower in MS patients. CSF and plasma nitrate levels did not correlate with age at onset and duration of the disease in the patient group. These data suggest that measurement of CSF levels of nitrate is not a marker of the activity of MS.
We compared CSF and serum levels of iron, copper, manganese, and zinc, measured by atomic absorption spectrophotometry, in 37 patients with Parkinson's disease (PD) and 37 matched controls. The CSF levels of zinc were significantly decreased in PD patients as compared with controls (p < 0.05). The serum levels of zinc, and the CSF and serum levels of iron, copper, and manganese, did not differ significantly between PD-patient and control groups. There was no influence of antiparkinsonian therapy on CSF levels of none of these transition metals. These values were not correlated with age, age at onset, duration of the disease, scores of the Unified Parkinson Disease Rating Scale of the Hoehn and Yahr staging in the PD group, with the exception of CSF copper levels with the duration of the disease (r = 0.38, p < 0.05). These results suggest that low CSF zinc concentrations might be related with the risk for PD, although they could be related with oxidative stress processes.
INTRODUCTION: Paraneoplasic cerebellar degeneration is seen clinically as a pancerebellar condition which is usually symmetrical. Different families of tumours are associated with this, particularly (in view of its frequency) oat cell pulmonary carcinoma, gynecological tumours and Hodgkin's lymphoma. CLINICAL CASE: Signs of cerebellar atrophy were seen on MR and cortical hypoperfusion was seen on Single Photon Emission Computerized Tomography (SPECT). We present the case of a 76 year old woman who presented with an asymmetrical pancerebellar disorder of gradual onset, with positive anti-self antibodies and undifferentiated carcinoma of the breast. CONCLUSIONS: Paraneoplasic cerebellar degeneration should be suspected in a patient with symmetrical, progressive cerebellar disease. The syndrome characteristically starts with a slightly uncoordinated gait. This progresses over a period of weeks or months to an ataxic gait with incoordination of the limbs, dysarthria and frequently nystagmus with oscilloscopy. No satisfactory treatment has been found for DCP in spite of trials with vitamins, corticosteroids, plasmapheresis and immunoglobulin infusion. Slight improvement may be seen after treatment of the primary tumour.
INTRODUCTION: Neurological involvement in no-Hodgkin lymphoma is usually a serious problem leading to subacute compression of the spinal cord secondary to invasion of the spinal extradural space. This condition may be the presenting form of the illness, although relatively infrequently. Onset with involvement of the cauda equina or as an isolated radiculopathy is even rarer and has mainly been described at lumbar level. CLINICAL CASE: We present the case of a 27 year old man with no significant previous clinical history who had progressive, insidious onset of moderate-serious senso-motor radiculopathy at C8. On MR there was an infiltrating mass affecting the nerve root and reaching the spinal extradural space. Following decompression laminectomy the patient's leg recovered and on histology there was a large cell B lymphoma. The other complementary tests showed disseminated disease, although analysis of the cerebro-spinal fluid (including cytology) was normal. HIV seriology was also normal. CONCLUSION: Although isolated nerve root involvement is unusual in the presentation of lymphoma it should be considered to be part of the differential diagnosis of nerve root syndromes in young patients with no history of trauma or degenerative lesions.
INTRODUCTION: Amyotrophic neuralgia is characterized by pain of acute or subacute onset, accompanied by weakness and occasionally by atrophy of the brachial muscles, of unknown origin. We present our experience over the past 20 years. PATIENTS AND METHODS: We made a retrospective review of 37 patients with the above diagnosis, following the criteria of other series of such cases published in the literature. RESULTS: Twenty four of the patients were men and thirteen were women. The average age was 38 (11 to 71). A relevant clinical history was recorded in 9 cases; infection (5), surgery (4), remote trauma (3) and vaccination (1). There was a painful onset of the condition in 32 patients; objective weakness of the superior brachial plexus (30), inferior (5) or both (2). Atrophy was present in 23 and hypoaesthesia in 13. Two patients had fasciculations and 9 had hyperreflexia. In all patients electromyographic studies showed a neurogenic pattern of denervation of the muscles clinically affected. The severity of the condition was divided into mild (18), moderate (16) and intense (3). Prognosis was good in 24 and sequelae remained in 11. There were 2 bilateral cases and 2 relapses but no familial cases. CONCLUSIONS: There was a ratio of men/women of 1.8:1 and onset usually when the patient was in his forties. Mild infection, surgery, remote trauma and vaccination were the commonest clinical factors. Onset was painful in 85%. Muscular weakness was predominantly in the superior brachial plexus (85%), followed by atrophy in 62%. There was hypoaesthesia in a third of the patients. Most cases were mild (50%) and made a complete recovery (70%). Our findings are similar to those described in most series in the literature.
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