["Telegraphist's " stroke].
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Biomedical subjects
Publications and source records attributed to A Berbel.
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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.
We measured total tau protein concentrations in the cerebrospinal fluid (CSF) of 26 non-demented Parkinson's disease (PD) patients and 25 matched controls. When compared with controls, PD patients had similar CSF tau protein concentrations. These values were not correlated with age, age at onset of PD, duration of PD, scores of the Unified PD Rating Scale (UPDRS), and the Hoehn and Yahr staging, and were not influenced significantly by antiparkinsonian drugs. Our results suggest that CSF tau protein levels are apparently unrelated to the risk of PD.
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INTRODUCTION: Lhermitte's sign was first described by Pierre Marie and Chatelin in 1917. Lhermitte published his report in 1920 and reviewed this in 1924. This phenomenon is characterized by the occurrence of an electric shock-like sensation going along the spine in a cervico-caudal direction with flexion of the neck, and may also be felt in the upper and lower limbs. Clinical cases. Case 1. A 49 year-old woman diagnosed as having breast cancer and being treated with cisplatin presented with Lhermitte's sign. On physical examination the osteotendinous reflexes were absent but the abdominal cutaneous reflexes were present. There was reduced sensitivity to vibration. Case 2. An 80 year-old man, previously operated on for adenocarcinoma of the colon, without further treatment, presented with progressive weakness of all four limbs and Lhermitte's syndrome. On examination there was obvious weakness of all four limbs, with the sensory level at C3. A cervical MR scan showed a metastasis in the vertebral body of C3 with epidural involvement and compression of the spinal cord. Case 3. A 54 year-old man was being treated by radiotherapy for cancer of the larynx. He presented at the onset of Lhermitte's sign but MR and physical examination were normal. CONCLUSION: Lhermitte's sign is non-specific, although in oncological patients a detailed clinical history and examination should be done seeking data regarding chemotherapy, radiotherapy and spinal compression.
INTRODUCTION: Idiopathic herniation of the spinal cord is a rarely diagnosed condition. It is important since this cause of paraparesis is potentially curable if diagnosis is made early. Our aim is to report a new case, review the relevant literature, describe the radiological findings and consider the etiopathogenic findings. CLINICAL CASE: We report the case of a 56 year old man with increasing difficulty in walking attributed to stenosis of the lumbar spinal canal, which did not improve after laminectomy. On examination he had sensory and motor deficits compatible with the Brown-Séquard syndrome. Dorsal MR showed ventral displacement of the thoracic spinal cord with disappearance of the anterior subarachnoid space together with a posterior arachnoid cyst at the same site. A further operation was done at the same place and the cyst removed. However, it was impossible to free the spinal cord since there was severe spinal arachnoiditis. The patient made little improvement. CONCLUSIONS: Herniation of the spinal cord is a rarely diagnosed condition. After careful study of the literature it seems that all patients present in a similar manner. Clinically there is a disorder of gait compatible with the Brown-Séquard syndrome. On MR there is sudden ventral displacement of the thoracic spinal cord, associated in some cases with an arachnoid cyst. When surgery is effective there is great improvement, so this diagnosis should be remembered in all cases of progressive paraparesis.
INTRODUCTION AND OBJECTIVES: Thalamic dementia is the clinical consequence of a disorder of both thalami. It is generally secondary to bilateral paramedial thalamic infarcts due to disorders of small blood vessels or cardioembolism. We report a case of dementia of acute onset involving the left thalamus and the genum of the right internal capsule. CLINICAL CASE: A 33 year old man, HIV positive, category B2, admitted to hospital for tuberculous meningitis presented with the acute onset of somnolence, followed by marked bradypsychism, personality changes, marked disorder of executive explicit memory without associated praxic, gnosic or language disorders. Ocular motility remained normal. There was left central facial paralysis with inverse emotive voluntary dissociation. The other cranial nerves were normal. There was left hemiparesia with extensor plantar reflex. No other alterations. Cerebral MR imaging was compatible with paramedial infarcts of the left thalamus and genum of the right internal capsule. CONCLUSIONS: Thalamic dementia generally occurs in bilateral paramedian thalamic disorders. There are cases of disorders of executive memory secondary to infarcts of the genum of the internal capsule due to interruption of the thalamotemporal pathways and a contralateral paramedial thalamic lesion.
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