Biomedical subjects
Publications and source records attributed to F Regli.
The effects of alcohol on the central nervous system can be subdivided into three main categories: the effects of acute intoxication (drunkenness, acute encephalopathy, stroke), the effects of tolerance and ethanol withdrawal (delirium tremens, seizures) and the delayed manifestations of chronic alcohol consumption (cerebellar degeneration, Wernicke's encephalopathy, dementia).
Three patients with mental neuropathy ("numb chin syndrome") secondary to systemic cancer are described. Two patients had malignant lymphoma and one a probable hypernephroma. In 2 patients, facial numbness was a presenting symptom of the malignancy. Investigations disclosed metastatic lesions of the mandible in one patient and cerebrospinal fluid was normal in 2 patients. Two patients died at 1 and 8 months respectively after onset of the neuropathy. Although benign in appearance, numb chin syndrome is most often, and sometimes initially, associated with visceral neoplasia or malignant hemopathy. It is a factor of poor prognosis. The pathogenesis is related either to metastasis of mandible, or neoplastic infiltration of the nerve itself.
Though the role of cerebral ischemia as an etiologic factor for epilepsy is accepted, the effect of seizures on stroke sequelae has received little attention. We describe 10 patients with poststroke partial epileptic seizures that were followed by persistent worsening of the previous neurologic deficit. Of 38 other patients with poststroke seizures who were examined during the same period, eight suffered transient neurologic worsening (Todd's phenomenon). Persistent worsening was associated with longer seizures and longer partial seizures before generalization. Risk factors, age, sex, other seizure features, and characteristics of previous stroke were irrelevant to developing persistent worsening of stroke sequelae. None of the patients with persistent worsening showed a new lesion or an extension of the previous ischemic area on computed tomography or magnetic resonance imaging, except one who had a first hemorrhage that spared the cortex and who suffered a second hemorrhage, which was lobar. Persistent worsening of a neurologic deficit following a seizure in patients with previous stroke may not be uncommon and may be due to a direct effect of the seizure itself on the infarcted area.
Seventeen patients with hemiataxia as a manifestation of thalamic infarction were studied. Hemiataxia had the main clinical characteristics of a "cerebellar type" of ataxia, though it never occurred in isolation, being associated with ipsilateral sensory disturbance (hemiataxia-hypaesthesia) in 7 patients, with ipsilateral sensory disturbance and hemiparesis (hypaesthetic ataxic hemiparesis) in 8 patients, and with hemiparesis (ataxic hemiparesis) in 2 patients. Recovery was good, and in all patients the sensory and motor disturbances improved or cleared before the hemiataxia. All patients had an infarct involving the lateral part of the thalamus (thalamogeniculate territory in 16, tuberothalamic territory in 1), also affecting the posterior limb of the internal capsule (PLIC) in 7 patients. Hemiataxia seemed linked to involvement of the caudal part of the ventral lateral nucleus of the thalamus or the immediately adjacent medial part of the PLIC. These structures are near the corticospinal pathways and the ventral posterior nucleus of the thalamus, explaining why hemiataxia is associated with hemiparesis or hypaesthesia in this type of infarct.
BACKGROUND: Diffuse uveal melanocytic proliferation is a rare paraneoplastic syndrome resulting in rapid bilateral visual loss due to proliferation of benign melanocytes within the choroid and ciliary body. Most of the previously reported cases have been seen with bilateral involvement and typical ocular features. PATIENT: The authors report the case of a 61-year-old man who presented with uniocular posterior pole lesions at the level of the retinal pigment epithelium and subsequently developed the typical bilateral lesions of diffuse uveal melanocytic proliferation. His clinical course was typical, with visual disturbance preceding signs and symptoms of malignancy by 5 months. Rapid decline ensued, and he eventually died 10 months after the onset of visual symptoms. RESULTS: Results of ocular pathologic examination showed the typical choroidal thickening due to the proliferation of melanocytes and the primary tumor was found to be an undifferentiated adenocarcinoma originating in either the pancreas or the esophagus. CONCLUSIONS: The very early funduscopic and fluorescein angiographic findings of diffuse uveal melanocytic proliferation are presented as well as the evolution, ocular pathology, and possible mechanisms for its development.
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We report the case of a woman who developed chronic meningitis and a brain abscess due to Mycobacterium avium, without immunosuppressed state. She was treated with antituberculous drugs but the infection progressed and she died 4 years after first becoming ill. The occurrence of CNS infection with M. avium in the absence of acquired immunosuppression is rare. Its diagnosis is difficult, delaying clinical recognition and appropriate therapy.
Three unusual patients who developed subacute facial numbness as the heralding symptom of an expanding tumor that involved the trigeminal nerve fibers are reported. The first patient had clinical and electrophysiological evidence of an isolated mental neuropathy as a result of metastatic lesions with bone destruction from a renal cell carcinoma. The second patient had a sensorimotor trigeminal neuropathy caused by a direct compression of the semilunar ganglion by a cavernous hemangioma of Meckel's cave. The last patient experienced facial numbness as the unusual presenting manifestation of a primary brainstem lymphoma. Patients 1 and 3 died a few weeks after the admission, whereas patient 2 poorly recovered. Despite the availability of new techniques for early diagnosis, this report demonstrates how difficult it can initially be to differentiate a 'benign' trigeminal neuropathy from serious conditions and underscores the poor prognosis of fifth nerve fibers involvement by an expanding mass. Early referral with clinical and electrophysiological evaluation appears to be of crucial importance.
The centrum ovale, which contains the core of the hemispheric white matter, receives its blood supply from the superficial (pial) middle cerebral artery (MCA) system through perforating medullary branches (MBs), which course toward the lateral ventricles. Though vascular changes in the centrum ovale have been emphasized in dementia, stroke from acute infarction in the centrum ovale is less well documented. We studied 36 patients with infarct limited to MB territory, without involvement of the lenticulostriate territory. Ten patients had a large infarct, associated with severe disease of the ipsilateral carotid artery and with neurologic-neuropsychological impairment not different from that of large MCA infarcts. In 26 patients, the infarct was small and round or ovoid, and was associated with hypertension or diabetes and with "lacunar syndromes," usually of progressive onset. These findings show that two forms of centrum ovale infarcts can be delineated according to infarct size and shape, clinical picture, risk factors, and associated vascular disease. We propose to classify subcortical infarcts in the carotid system into four main territory groups: (1) deep perforator territory (from the MCA trunk, carotid siphon, anterior choroidal artery, anterior cerebral artery trunk, Heubner's artery, and posterior communicating artery); (2) perforating MB territory (from the superficial MCA branches); (3) junctional (territory between 1 and 2); and (4) combined territories.
We studied the correlations between the pattern of weakness, stroke type, topography, and etiology in 255 patients whose first stroke was manifested by isolated hemiparesis. They represented 14% of consecutively admitted stroke patients. The weakness distributions were as follows: face, upper limb, and lower limb (FUL) (50%); face and upper limb (FU) (29%); upper limb (U) (10%); and upper and lower limb (UL) (9%). Twenty-nine percent of the patients had dysarthria, which was of no localizing value. Less than one half of the patients had a deep infarct, and one third had a potential embolic source from the heart or large arteries. Logistic regression analysis showed that history of hypertension and type of weakness distribution were the main factors accounting for lesion localization: patients with FUL distribution and hypertension had a 90% probability of deep infarct; patients either with FUL distribution but no hypertension or with UL distribution and hypertension each had 70% probability of deep infarct. Pure motor monoparesis was almost never caused by a deep infarct. We suggest that the assumption of a lacunar etiology to a pure motor stroke should be applied only to patients with FUL involvement.
A 49-year-old with long-standing hypertension and diabetes developed numbness and sensory loss over the left side of the body consistent with a diagnosis of Pure sensory stroke (PSS). However, CT showed a subcortical infarction in the middle cerebral artery (MCA) territory, which evolved a few hours later towards a large hemispheric infarction associated with severe neurologic worsening. Doppler ultrasounds showed ipsilateral carotid occlusion and contralateral severe stenosis. These findings suggest that PSS may sometimes herald large infarction in the MCA territory in association with carotid occlusion.
We report here two patients presenting a myopathy associated with a monoclonal gammopathy (of IgG lambda or IgA kappa type) and, in one case, also with a leukoencephalopathy. Possible etiopathological mechanisms between these neurological diseases and an immune disregulation are discussed.
Four patients with multiple sclerosis presented with pseudoradicular limb pain. There was a limb weakness with sensory loss and areflexia. Magnetic resonance imaging showed a demyelinating plaque in the cervical cord with a characteristic T2 relaxation time. A direct relationship between the plaque and the clinical troubles was established in all cases. Treatment with corticosteroids was helpful, and other treatments (carbamazepine) were not necessary. The mechanisms of such pain are unknown but some of the symptoms may be the result of lesions in the endogenous pain control system.
A case study of acute ischemic monomelic neuropathy due to occlusion of the right subclavian artery is reported. The occlusion was embolic. Clinical examination showed sensory loss and severe distal weakness of the limb without obvious muscle necrosis. EMG showed motor and sensory nerve axon loss, chiefly distally. Despite surgical intervention, the neurological damage was irreversible.
Three cases of bilateral deafness with cytologically-demonstrated meningeal carcinomatosis are reported. The first patient, a 64-year old man, presented with bilateral deafness, gait disturbances, and bilateral facial paresis. The second patient, a 78-year-old man, had bilateral deafness, unsteady gait and fluctuations in consciousness. The last patient, a 69-year-old man, complained of bilateral deafness and severe headache, and presented with right facial paresis and left laterodeviation while walking. All three patients had abnormal cochleo-vestibular findings and brainstem auditory evoked responses (BAER) that suggested peripheral lesions with absent or very delayed I waves. The brain CT scans with an without contrast enhancement were entirely normal, and the diagnosis was established by lumbar puncture. From our own cases and a review of the literature, deafness in meningeal carcinomatosis may start unilaterally but becomes bilateral in less than a week. Vestibular disturbances may not be apparent, but they can be demonstrated in almost all cases. Facial paresis or plegia is also a very frequent finding. The destruction of the eighth and seventh cranial nerves is probably due to direct infiltration by neoplastic cells as well as to ischemia through compression of the nerve supplying vessels. The 3 cases presented here emphasize once again the important fact that meningeal carcinomatosis remains a cytological diagnosis, several lumbar punctures being sometimes necessary, and that cerebrospinal fluid studies cannot yet be supplanted by other diagnostic techniques like contrast-enhanced CT or MRI with gadolinium.
We present clinical date and follow up in a rehabilitation unit with a multidisciplinary team for twenty two old patients with severe cerebrovascular disease. The patients were fully examined in the "Service de neurologie de Lausanne". New cerebral vascular accidents occurred in three patients. The other patients improved in functional status evaluated by the Barthel-Index in spite of no significant changes in neurological and neuropsychological status.
UNLABELLED: A retrospective study of patients who suffered cerebrovascular accidents (CVA particularly brainstem damage) was conducted to look for risk factors which, present on admission, indicated the chance of survival. The evolution of 100 patients, hospitalized in the Neurology Department, University Hospital Lausanne, after having had their first CVA was studied. The risk factors chosen were age, sex, hypertension, smoker, late onset diabetes, hypercholesterolemia as well as the conscious state, the location of the lesion and whether it was bi- or unilateral. Analysis of the data was performed by means of a stepwise multiple logistic regression program. The results demonstrated that diabetes or bilateral lesions, regardless of their position in the brainstem, or the combination of these two factors, indicated an unfavorable outcome, associated with a short term mortality rate greater than 30%. In their absence and despite the presence of the other risk factors, it was less than 2%. At one month the mortality rate was shown to be very high. At two month it was non insignificant and thereafter it became similar to that of the Swiss population. Brainstem lesions extending to the vital centres or respiratory complications related to the state of dependence were the most common causes of short term deaths. At long term the aetiology of death was the same as for the population as a whole. CONCLUSION: The results obtained show the importance of the prevention and treatment of patients at risk of CVA, particularly diabetes. To avoid the fatal complications, that arise from severe handicap in bedridden patients, the management must be rapid and efficient (physiotherapy and drugs).
The use of new cardiopulmonary resuscitation techniques has considerably increased the possibility of survival after cardiopulmonary arrest. It is of considerable interest to evaluate the extent of any anoxic brain damage. The EEG has proven very informative in the assessment of cerebral function in patients with anoxic encephalopathy. Its role is unchanged despite advanced imaging techniques. In 75 patients, the value of EEG recorded on the days after the onset of disturbances has been compared with the initial Glasgow coma scale (GCS) as a predictor of short-term prognosis. The EEG patterns were classified into 6 major grades and 12 sub-grades which were defined on the basis of the morphology of dominant activities, distribution and reactivity to external stimuli. A favorable outcome occurred with grade I to IIB associated with a GCS of 5 above. A bad prognosis was suggested by grades IVA to VC, particularly when epileptiform discharges were present, and the GCS was always below 5 in these patients. With new EEG grading scales in anoxic encephalopathies, the accuracy for predicting survival (or death) is now 90%.