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Biomedical subjects

F Regli

Publications and source records attributed to F Regli.

At least 73 records · Page 4Linked to original sources
PubMed1994

The "jerky dystonic unsteady hand": a delayed motor syndrome in posterior thalamic infarctions.

We report the cases of three patients with a thalamic infarct in the territory of the posterior choroidal artery involving the posterior thalamic nuclei. These patients developed delayed complex hyperkinetic motor syndromes, associating ataxia, tremor, dystonia, myoclonus and chorea, which we call "the jerky dystonic unsteady hand". One patient had a severe myoclonic and ataxic-dystonic choreoathetosis; another showed a so-called rubral tremor (myoclonic ataxia with resting, action, and wing-beating tremor) with dystonia; and the third one had a dystonic and ataxic hand with intermittent mild action myoclonus. All of them had sensory dysfunction; two had also presented with a painful Dejérine-Roussy syndrome. All had CT or MRI-proven infarcts in the territory of the posterior cerebral artery involving the posterior choroidal territory with an abnormal signal in the posterior area of the thalamus (pulvinar nucleus) but sparing the other thalamic, subthalamic and midbrain structures. These delayed myoclonic complex hyperkinetic syndromes have not been reported before, and we did not observe them in other topographic forms of thalamic infarcts. They may thus represent a new entity of movement disorders due to lesions in the posterior thalamic nuclei, with specificity for posterior choroidal artery infarcts.

Adult↗
PubMed1994

Pure amnesia after unilateral left polar thalamic infarct: topographic and sequential neuropsychological and metabolic (PET) correlations.

A 54-year-old patient who had an isolated small polar thalamic infarct and acute global amnesia with slight frontal type dysfunction but without other neurological dysfunction was studied. Memory improved partially within 8 months. At all stages the impairment was more severe for verbal than non-verbal memory. Autobiographic recollections and newly acquired information tended to be disorganised with respect to temporal order. Procedural memory was unaffected. Both emotional involvement and pleasure in reading were lost. On MRI, the infarct was limited to the left anterior thalamic nuclei and the adjacent mamillothalamic tract. The regional cerebral metabolic rate of glucose (measured with PET) was decreased on the left in the thalamus, amygdala, and posterior cingulate cortex 2 weeks after the infarct, and in the thalamus and posterior cingulate cortex 9 months later. These findings stress the specific role of the left anterior thalamic region in memory and confirm that longlasting amnesia from a thalamic lesion can occur without significant structural damage to the dorsomedial nucleus. Furthermore, they suggest that the anterior thalamic nuclei and possibly their connections with the posterior cingulate cortex play a role in emotional involvement linked to ipsilateral hemispheric functions.

Amnesia↗
PubMed1994

Infarcts in the territory of the lateral branch of the posterior inferior cerebellar artery.

The territory of the lateral branch of the posterior inferior cerebellar artery (1PICA) supplies the anterolateral region of the caudal part of the cerebellar hemisphere. Because infarcts in the territory of the 1PICA have rarely been studied specifically, 10 patients with this type of infarct are reported. An 1PICA infarct was isolated in only three patients, whereas it was associated with brainstem infarct in four, with occipital infarct in one, and with multiple infarcts in two patients. The most common symptom at onset was acute unsteadiness and gait ataxia without rotatory vertigo (six patients). Unilateral cerebellar dysfunction was found in all patients, with limb ataxia (nine patients), dysdiadochokinesia (five patients), and ipsilateral body sway (four patients), but dysarthria and primary position nystagmus were notably absent. In the patients with a coexisting infarct in the brainstem, cranial nerve and sensorimotor dysfunction was prominent and often masked the signs of cerebellar dysfunction. Unlike other infarcts in the PICA territory, 1PICA territory infarcts were mainly associated with vertebral artery atherosclerosis (six patients), whereas cardiac embolism was less common (three patients). Unilateral limb ataxia without dysarthria or vestibular signs suggests isolated 1PICA territory infarction and should allow its differentiation from other cerebellar infarcts.

Aged↗
PubMed1994

Justification of hospital days and epidemiology of discharge delays in a department of neurology.

We have developed a protocol to identify unnecessary days of hospitalisation in the Department of Neurology of the Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. Seventy-four parameters (medical, social, type of investigation and treatment, degree of disability and of dependence) potentially associated with the length of stay were studied prospectively in 511 nonselected patients consecutively admitted to the Department over a period of 5 months. Each day spent on the wards was analyzed on a day-to-day basis and was classified into one of two groups: those due to a medical reason (4,700 hospital days), and those due to a nonmedical reason (1,184 days). These delays resulted chiefly from difficulties in obtaining laboratory investigations, especially in patients who were not disabled and who had been admitted for investigations (3.8% of hospital days, compared to 1.5% for patients with severe or total dependence) or from awaiting transfer to either another department or a nursing home. This second cause of delay markedly increased the length of stay of patients with severe or total dependence and with limited mobility on the first day (26.0 days, compared with 7.4 days for nondisabled patients) and, above all, on the last day spent in our Department (27.0 days, compared with 8.0 days). The ongoing analysis of these data may provide information as to which parameters could be influenced by neurologists in order to reduce the length of stay in hospital.

Adolescent↗
PubMed1994

Pure midbrain infarction: clinical syndromes, MRI, and etiologic patterns.

We studied 22 patients with first stroke and infarct limited to the midbrain on MRI. We selected these patients (8%) from 281 with posterior circulation infarct admitted consecutively into a primary care center. All patients underwent a systematic protocol of investigations including MR imaging and angiography, and echocardiography. Most infarcts fitted well to arterial territories drawn in preestablished templates. Middle midbrain involvement was the most common, mainly in the paramedian territory supplied by the basilar artery. Infarct in the mesencephalic territory of the posterior cerebral artery was less common, while superior cerebellar artery territory infarct was extremely rare, and posterior choroidal artery territory infarct did not occur. The neurologic picture was dominated by eye-movement disorders. Patients with isolated upper or lower midbrain infarct had no localizing clinical findings, but patients with middle midbrain infarct had a localizing picture mainly with nuclear or fascicular third nerve palsies that commonly developed in isolation. Vertical gaze paresis, pure motor hemiparesis, four-limb ataxia from unilateral lesion, and hypesthetic ataxic hemiparesis also occurred. Contrary to a common view, cardioembolism was not a more common etiology than basilar artery stenosis or small-vessel disease.

Aged↗
PubMed1994

Acute reversible ataxo-myoclonic encephalopathy with flecainide therapy.

A 73 year-old patient with Wolff-Parkinson-White syndrome and paroxysmic supraventricular tachycardia developed an acute reversible encephalopathy within 15 days of initiation of flecainide. The clinical picture was characterized by visual hallucinations, agitation, anxiety, but no disorientation, and a severe cerebello-myoclonic syndrome with total inability to stand up and walk, which was fully reversible on discontinuing the medication.

Aged↗
PubMed1994

Segmental pruritus and intramedullary vascular malformation.

Pruritus remains a medical mystery. Generally related with dermatological diseases, it is rarely associated with a neurological cause. We report a case of segmental pruritus (right T2 dermatome) related to an intramedullary vascular malformation. An anatomicophysiological explanation is presented. It is the first demonstration in man that pain receptors are not involved in pruritus, and suggests pruritus is under the control of descending inhibitory pathways.

Aged↗
PubMed1994

Autoantibodies in neurological paraneoplastic diseases.

Paraneoplastic syndromes such as the subacute sensory neuronopathy (SSN) and paraneoplastic cerebellar degeneration (PCD) are associated with autoantibodies directed against various neural antigenic structures. SSN is characterised by autoantibodies against a neuronal intranuclear component called Hu (M. W. 35 to 40 kD), whereas in PCD these antibodies are directed against a Purkinje cell cytoplasmic component called (M. W. 34 and 65 kD). Neuroblastoma cell lines maintained in culture have been shown to contain neuronal antigens. We have demonstrated the presence of the Hu antigen in neuroblastoma cell lines such as SKN-SH, LAN-1 and IMR-32 by both immunocytochemistry and immunoblots of nuclear extracts. The Yo paraneoplastic antigen has been found to be expressed in HeLa cells. These methods may be used for screening of patients with suspected paraneoplastic disease and/or malignancy. Western blot analysis appears to be superior to immunohistochemistry alone. In our experience only 11 out of 122 SCLC patients were positive for the anti-Hu antibody, whereas 5 out of 5 patients with SSN/SCLC were positive, and all of the neurological controls were negative. The availability of cell lines expressing paraneoplastic antigens offers an easy diagnostic assay which may complement or replace conventional immunohistochemistry.

Autoantibodies↗
PubMed1994

[Optic neuropathy caused by alcoholism and smoking: a diagnostic pitfall of Leber's optic neuropathy].

A 39 year-old patient suffered bilateral painless visual loss while consuming abusively alcohol and cigarettes. Family history was remarkable for a similar episode in the patient's brother 21 years ago, while drinking and smoking heavily. A diagnosis of toxic optic neuropathy was made in another institution, but due to the positive family history, mitochondrial DNA was studied for a point mutations associated with Leber's hereditary optic neuropathy. A homoplasmic mutation at nucleotide 11778 was found. The patient's brother could not be studied. This case emphasizes the probable deleterious effects of epigenetic factors such as alcohol and cigarette smoke in patients harbouring mitochondrial DNA mutations associated with Leber's hereditary optic neuropathy and the need for mitochondrial DNA studies in atypical cases of optic neuropathy.

Adult↗
PubMed1994

[Autoimmune paraneoplastic cerebellar degeneration].

Paraneoplastic cerebellar degeneration is a well-defined clinical entity typically associated with ovarian or breast carcinoma. Serum or CSF of these patients has been shown to contain autoantibodies against the cytoplasm of Purkinje cells. These antibodies have been referred to as anti-Yo. We report the case of a 67-year old woman with ovarian adenocarcinoma presenting with subacute cerebellar dysfunction. Her serum was found to react with the cytoplasm of Purkinje cells and HeLa cells. On Western blot analysis a 60 to 70 kD reaction was obtained. This suggests the presence of a classical anti-Yo antibody.

Adenocarcinoma↗
PubMed1993

[Subcortical cerebrovascular accidents: correlation between clinical aspects, topography and flow measurements using single photon emission tomography].

35 patients with subcortical ischemic or hemorrhagic stroke included in the Lausanne Stroke Registry underwent cerebral blood flow measurement using I-123-IMP and 99mTc-HMPAO single photon emission tomography (SPECT). Our findings suggest that neuropsychological disturbances are not systematically correlated to cortical hypoperfusion secondary to the deep lesion (diaschisis). The role of cortical diaschisis was especially prominent in patients with aphasia and left subcortical lesion.

Adolescent↗
PubMed1993

[Tremors].

Tremor can present a diagnostic problem in family practise. Systematic analysis of this symptom allows rapid classification of the problem without use of rigid definitions. Unilateral or very asymmetric tremor that disappears at the onset of a movement is for example a typical sign for parkinsonism without analysis and differentiation between tremor at rest or position-dependent tremor. Also one may assume, that a symmetric tremor that aggravates during standing or movement in most cases is essential and not of cerebellar origin. Different forms of tremor and their therapies and main tremor types are presented graphically.

Adult↗
PubMed1993

[Neurologic polymorbidity in senescence: 467 cases].

In this paper we studied the neurological polymorbidity in a group of 467 hospitalized patients of more than 70 years of age. A disorder of the central nervous system (CNS) was of the principal mainly diagnosis in 393 cases, the peripheral nervous system (PNS) in 71 cases and in 3 cases a myopathy. In 77.9%, the various neurological disorders were within the CNS, including mainly cerebrovascular events (226 cases, 48%). Among 156 patients, there was an accompanying disorder of the PNS.

Aged↗