Search PubMed⌕ Search

Biomedical subjects

F Regli

Publications and source records attributed to F Regli.

At least 37 records · Page 2Linked to original sources
PubMed1997

[Olivo-ponto-cerebellous degeneration. A study of 21 patients defined by the Quinn criteria].

21 patients with diagnosis of idiopathic OPCA were examined clinically and evaluated by MRI or CT-scan. On the basis of Quinn's criteria for MSA, patients were subdivided into those with probable MSA (48 p. cent) and those with possible MSA (52 p. cent). Median age at onset was 51.8 years. The initial clinical feature of the disease was ataxia, but the presence of multiple system involvement was clear in all cases. The combination of involvement of four different system (cerebellar, parkinsonian, pyramidal, autonomic) was the most common (28.5 p. cent), followed by the association of cerebellar and pyramidal features (24 p. cent). Autonomic symptoms were present in 48 p. cent of patients. CY-scan or MRI showed cerebellar and brainstem atrophy in 43 p. cent of cases. There was no relation with the duration of the disease or the severity of clinical features. Moreover brainstem auditory evoked response and EMG were not helpful in diagnosis.

Cerebellar Ataxia↗
PubMed1996

Gene therapy for amyotrophic lateral sclerosis (ALS) using a polymer encapsulated xenogenic cell line engineered to secrete hCNTF.

The gene therapy approach presented in this protocol employs a polymer encapsulated, xenogenic, transfected cell line to release human ciliary neurotrophic factor (hCNTF) for the treatment of Amyotrophic Lateral Sclerosis (ALS). A tethered device, containing around 10(6) genetically modified cells surrounded by a semipermeable membrane, is implanted intrathecally; it provides for slow continuous release of hCNTF at a rate of 0.25 to 1.0 micrograms/24 hours. The semipermeable membrane prevents immunologic rejection of the cells and interposes a physical, virally impermeable barrier between cells and host. Moreover, the device and the cells it contains may be retrieved in the event of side effects. A vector containing the human CNTF gene was transfected into a line of baby hamster kidney cells (BHK) with calcium phosphate using a dihydrofolate reductase-based selection vector with a SV40 promoter and contains a HSV-tk killer gene. hCNTF is a potent neurotrophic factor which may have utility for the treatment of ALS. Systemic delivery of hCNTF in humans has been frustrated by peripheral side effects, the molecule's short half life, and its inability to cross the blood-brain barrier. The gene therapy approach described in this protocol is expected to mitigate such difficulties by local intrathecal delivery of a known quantity of continuously-synthesized hCNTF from a retrievable implant.

Amyotrophic Lateral Sclerosis↗
PubMed1996

Hashimoto's myoclonic encephalopathy: an underdiagnosed treatable condition?

We report two patients with subacute diffuse encephalopathy characterized by confusion, myoclonic encephalopathy, and mild akineto-rigid extrapyramidal signs in one case and by apathy, memory deficit, and partial complex seizures in the other. Hashimoto's thyroiditis with high titers of anti-thyroglobulin antibodies was diagnosed in both patients, who were unresponsive to anticonvulsant medication, but showed rapid neurological improvement following steroid treatment. On neuropsychological examination, predominant frontotemporal dysfunction was noted. Electroencephalographic activity was remarkable for its rhythmical delta activity, unresponsive to, or even paradoxically increased by, anticonvulsant treatment. On magnetic resonance imaging, atrophy with temporal predominance was found. These observations support the idea that this potentially treatable dementia and movement disorder should be classified as a separate clinical entity.

Adult↗
PubMed1996

"Echoing approval": a new speech disorder.

We report the cases of two patients presenting a peculiar speech disorder, which we have named "echoing approval", in which the patients echo, in replying to questions in a dialogue with short phrases, the positive or negative syntactical construction of a question, or its positive or negative intonation, but without any repetition of whole or part of sentences. When asked about their symptoms, the patients replied 80% of the time with "yes, yes", "that's right", or "exactly" to positive questions and "no, no" or "absolutely not" to negative questions, regardless of their actual symptoms and oblivious to self-contradiction. In addition, when the examining doctor was speaking to a medical colleague in the patient's presence and using medical terminology that the patient did not understand, he/she agreed or disagreed with any sentence and technical word uttered in a way entirely dependent on the syntax or intonation used. To distinguish this speech disorder from echolalia or verbal perseverations, with which it may be superficially confused, we suggest that it be called "echoing approval", as it may be part one of the manifestations of the environment-dependency syndrome. This clinical picture was found to be associated with features of transcortical motor aphasia and frontal lobe signs. One patient had a bilateral callosofrontal malignant glioma and the other a probable multiple system atrophy with global deterioration, pre-eminent frontal release signs, diffuse leukoencephalopathy and multiple lacunes. On the basis of these clinical deficits and neuroimaging features, we are unable to delineate the common, or minimal, lesioned network required for this symptomatology to occur, especially in the absence of a series of patients, and with such a difference in both the location and causes of the lesions. However, bilateral frontosubcortical dysfunction was pre-eminent in the clinical picture in both patients, even though more diffuse brain pathology was seen in one, and it might be speculated that dysfunction of the bilateral orbitofrontal and frontomesial motor frontosubcortical circuits might be involved in the aetiology of this peculiar speech disorder.

Aged↗
PubMed1996

Justification of hospital days and discharge delays in a non-selected population of acute stroke patients.

Using a published protocol, we evaluated a non-selected population of stroke patients to identify unnecessary days of hospitalisation in the Department of Neurology at the Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland. This study was undertaken to determine whether physicians can modify the non-medically justified hospital days. We prospectively studied 118 patients with stroke admitted to our Department over a period of 5 months. Each day spent on the ward was placed in one of two categories: those due to medical reasons (1,391 hospital days) and those due to non-medical reason (518 days). Using a previously published protocol, 74 parameters were evaluated. Delays in obtaining examinations or a specialist's consultation accounted for a small proportion of waiting days (1.9% of total hospital days), which was greater in patients who were not disabled (0.9% of hospital days) than in patients with total dependence (0.2% of hospital days). The delays resulted mainly from awaiting transfer either to another department or to a nursing home. The length of stay increased with severity of dependence. However, the number of days spent for transfer to a nursing home was also relatively high in the non-dependent patient group (42% of hospital days). This study demonstrates that neurologists cannot easily influence the length of stay in hospital. It also corroborates the need to develop short- and long-term chronic care facilities to facilitate the transfer of patients once there are no further medical reasons for staying in hospital.

Adolescent↗
PubMed1996

Intrathecal delivery of CNTF using encapsulated genetically modified xenogeneic cells in amyotrophic lateral sclerosis patients.

Neuronal growth factors hold promise for providing therapeutic benefits in various neurological disorders. As a means of ensuring adequate central nervous system delivery of growth factors and minimizing significant adverse side effects associated with systemic delivery methods, we have developed an ex vivo gene therapy approach for protein delivery using encapsulated genetically modified xenogeneic cells. Ciliary neurotrophic factor (CNTF) has been shown in various rodent models to reduce the motor neuron cell death similar to that seen in amyotrophic lateral sclerosis (ALS). The initial trials focusing on the systemic administration of CNTF for ALS have been discontinued as a result of major side effects, thus preventing determination of the potential efficacy of the molecule. In order to deliver CNTF directly to the nervous system, we conducted a phase I study in which six ALS patients were implanted with polymer capsules containing genetically engineered baby hamster kidney cells releasing approximately 0.5 microgram of human CNTF per day in vitro. The CNTF-releasing implants were surgically placed within the lumbar intrathecal space. Nanogram levels of CNTF were measured within the patients' cerebrospinal fluid (CSF) for at least 17 weeks post-transplantation, whereas it was undetectable before implantation. Intrathecal delivery of CNTF was not associated with the limiting side effects observed with systemic delivery. These results demonstrate that neurotrophic factors can be continuously delivered within the CSF of humans by an ex vivo gene therapy approach, opening new avenues for the treatment of neurological diseases.

Amyotrophic Lateral Sclerosis↗
PubMed1996

Multiple acute infarcts in the posterior circulation.

OBJECTIVE: to evaluate clinical, radiological, and prognostic features of patients with multiple acute infarcts in remote arterial territories of the posterior circulation. DESIGN: Data analysis from a prospective acute stroke registry in a community based primary care centre using a standard protocol including MRI and MRA. RESULTS: In three and a half years, 27 of the 236 patients (11%) with posterior circulation stroke had multiple acute infarcts in the posterior circulation as shown by gadolinium enhancement on MRI. Eighteen patients had multiple infratentorial and supratentorial infarcts including the cerebellum and posterior cerebral artery territory, with coexisting brainstem involvement in seven patients. Fourteen patients had a rostral basilar artery syndrome and cerebellar signs; four patients had a visual field defect with cerebellar signs. Causes were vertebral (six) or basilar (four) artery atheromatosis, and cardioembolism (four). Seven patients had multiple acute infarcts in the posterior circulation of the cerebellum and lower brainstem. Brainstem and cerebellar signs were found in most patients (five); aetiologies were small vessel disease (four), cardioembolism (one), and vertebral artery dissection (one). Two patients with large artery atheromatosis had multiple acute infarcts in the posterior circulation in the brainstem and posterior cerebral artery territory. One month after stroke more than 25% of the patients were dependent or had died. There was no difference in the outcome between the three groups, and recovery was linked to the size of infarcts rather than to a high number of infarcts. CONCLUSIONS: multiple acute infarcts in the posterior circulation usually involve the cerebellum. Simultaneous brainstem and posterior cerebral artery territory infarcts sparing the cerebellum are uncommon. They can be suspected clinically before neuroimaging, mainly when supratentorial and infratentorial infarcts coexist. This may be important, because different patterns of infarction are associated with different causes of stroke.

Activities of Daily Living↗
PubMed1996

Isolated infarcts of the pons.

We studied 36 patients with MRI-proven isolated acute pontine infarct. Corresponding to the constant territories of intrinsic pontine vessels, infarcts followed a predictable distribution, enabling us to delineate three main syndromes. Twenty-one patients had a ventral pontine infarct. Motor involvement varied from mild hemiparesis (ventrolateral pontine syndrome) to severe hemiparesis with bilateral ataxia and dysarthria (ventromedial pontine syndrome). In addition, three-fourths of the patients had clinical evidence for usually mild tegmental dysfunction. Eleven patients had a tegmental pontine infarct, presenting tegmental signs (eye movement disorders, cranial nerve palsies, sensory disturbances), and mostly mild motor deficits (tegmental pontine syndrome). Only four patients had alternating deficits, and these never corresponded to any of the so-called classic pontine syndromes. Infarcts in the medial and the extreme lateral tegmental territory were never observed in isolation, being always associated with cerebellar or larger (and multiple) infarctions in the posterior circulation. Four patients with a bilateral ventrotegmental pontine infarct presented with acute pseudobulbar palsy, bilateral motor deficits, and tegmental signs. The results of etiologic work-up emphasize the concept of basilar artery branch disease, which was the most common presumed cause of stroke (16/36, 44%). Basilar artery branch disease was particularly associated with large ventral infarcts, severe clinical symptomatology, progressive or fluctuating course, and local recurrence. Presumed small-artery disease (9/36, 25%) was usually associated with small ventral or tegmental infarcts and rapidly improving lacunar syndromes. Large-artery stenosis (8/36, 22%) and cardioembolism (1/36, 3%) were less common than in series of posterior circulation infarcts that include simultaneous pontine and extrapontine lesions. Recovery was good in two-thirds of the patients, the worse outcome being associated with large ventral infarcts.

Adult↗
PubMed1996

Unilateral spatial neglect recovery after sequential strokes.

We describe a patient who had sequential strokes in both hemispheres with a severe unilateral spatial neglect after a first right-sided parietal infarct and abrupt disappearance of the neglect after a second left-sided frontal infarct. The first lesion involve the caudalmost right angular gyrus (area 39), whereas the second lesion involved the left frontal eye field (area 8) and surrounding cortex. Those two cortical areas are assumed to have a pivotal role in modulating both shifts of attention within extrapersonal space and saccadic eye movements through their connections with subcortical structures, in particular, superior colliculi and thalamic nuclei. Our case supports the existence of a distributed anatomic-functional network in subserving directed spatial attention.

Aged↗
PubMed1996

Failure to recall (but not to remember): pure transient amnesia during nonconvulsive status epilepticus.

We report a patient with a generalized frontal-predominant nonconvulsive status epilepticus without clinically apparent altered consciousness. The patient was examined and EEG performed during and after the episode. Severe retrograde and anterograde amnesia during the seizure, contrasting with a preservation of ongoing memories formation that could be assessed only after its resolution, suggests a transient disconnection of access to stored representations. This unusual memory disorder is both clinically and electrographically dissimilar to other reported cases of transient epileptic amnesia. Although the patient probably had numerous episodes previously, there was no history of overt seizure.

Adult↗
PubMed1996

Prognosis after stroke followed by surgical closure of patent foramen ovale: a prospective follow-up study with brain MRI and simultaneous transesophageal and transcranial Doppler ultrasound.

BACKGROUND: The risk of stroke and the long-term prognosis of recurrent strokes in young patients with patent foramen ovale (PFO) are not well known. For this reason, the treatment of these patients remains empirical. An alternative treatment to prolonged antithrombotic therapy may be surgical closure of the PFO. METHODS: Thirty patients (20 men and 10 women) with stroke and PFO were prospectively selected among 138 patients with stroke and PFO for a study of surgical closure of PFO at our center. Eligible patients were < 60 years old, had negative results of a systematic search for another cause of stroke (first criterion), and met two of the four following criteria: (1) recurrent clinical cerebrovascular events or multiple ischemic lesions on brain MR, (2) PFO associated with an atrial septal aneurysm, (3) > 50 microbubbles counted in the left atrium on contrast transesophageal echocardiography (TEE), and (4) Valsalva maneuver or cough preceding the stroke. Patients selected in this manner for surgery were considered to be a subgroup with a higher risk of stroke recurrence. RESULTS: All patients had a direct suture of PFO while under cardiopulmonary bypass without recorded early or delayed significant complication. All patients underwent a new brain MRI and TEE simultaneous with transcranial Doppler ultrasonography after contrast injection at 8 +/- 3 months after surgery. After a mean follow-up of 2 years without antithrombotic treatment, no recurrent cerebrovascular event (stroke or transient ischemic attack [TIA]) and no new lesion on MRI had developed. Postoperative contrast TEE and transcranial Doppler ultrasonography showed that two patients had residual interatrial right-to-left shunting, although much smaller than before surgery, associated with single versus double continuous suture. CONCLUSIONS: Our study of 30 selected stroke patients with surgical suture of PFO showed a stroke recurrence rate of 0% and no significant complication. Residual right-to-left shunting may be avoided by double continuous suture of the PFO. In the absence of controlled studies to guide individual therapeutic decisions, our findings show that PFO closure can be done safely and may be considered to avoid recurrence in selected patients with long life expectancy and presumed paradoxic embolism.

Adult↗
PubMed1996

[Peripheral nerve and spinal cord complication in intravenous heroin addiction].

The neurological complications observed in 6 HIV negative intravenous drug users are reported. Four developed acute neuromuscular involvement in a lumbosacral or brachial distribution with rhabdomyolysis, myoglobinuria, hypovolemia, renal and hepatic failure in the 3 most severely affected patients. Despite evidence of immunologic abnormalities and especially presence of anti-heroin antibodies, we feel that causative mechanisms include mixed compression and ischemia with an underlying toxic myopathy, resulting in segmental myopathy with secondary compression of peripheral nerves. Two patients developed myelopathy with acute or chronic onset. The mechanisms were vascular with spinal cord infarction in the acute form and probably infectious with secondary compressive arachnoiditis in the chronic form. In these 2 patients with myelopathy, outcome was poor.

Acute Disease↗
PubMed1995

[Neurology at the threshold of the 21st century].

In the controversial field of a continuously changing neurology with the problem of rising medical costs, the perspectives of modern neurology at the beginning of a new century are described by four aspects: 1. Relatively new are the studies of neuropsychiatric symptoms in neurologic diseases. An immediate diagnosis and treatment of these symptoms can improve the outcome of the neurologic disease. 2. An example for the progress in investigative methods by medical technology is magnetic resonance imaging which allows a more refined diagnosis and which improves and reevaluates the importance of the clinical examination. There is a warning on the other hand about unnecessary and badly planned examinations. 3. Advances in clinical neurology are mentioned by describing neurogenetic diseases, infectious neurologic diseases and new forms of dementia. 4. Some therapeutical aspects concerning neuroprotection and neurologic intensive care are discussed. Finally, the aims for the training of future neurologists are briefly outlined.

Brain↗
PubMed1995

[Management of patients with simple cervical distortions].

Common distortion of the cervical spine without evidence of neurological or osteoligamentary damage is a frequent consequence of indirect head and neck trauma. The mechanism of the injury (called "whiplash", "coup de lapin", or "Schleudertrauma") does not imply direct trauma to the head or neck. In the acute phase, common distortion of the cervical spine requires treatment. Rapid management may avoid or considerably reduce the chronic pain syndrome with the characteristic chronic tension-like headache. The chronic pain and the numerous associated functional disorders are not well understood: certain factors favour central and peripheral dysfunction and others emphasize the importance of extra-trauma phenomena. In our opinion, extra-trauma phenomena would explain the invalidating nature of pain in a small number of patients. Management of these chronic patients requires a multidisciplinary approach aimed at helping the patient overcome the inconveniences of this condition.

Accidents, Traffic↗
PubMed1995

[Therapeutic immunomodulation in neurology: from hope to reality. Exemplified by multiple sclerosis].

Multiple strategies for specific therapies are presently attempted in multiple sclerosis, in which an autoimmune reaction leads to demyelinisation within the central nervous system. Similar approaches are generally used in other potential autoimmune diseases. They try to interfere with the presentation and recognition of putative autoantigens by the immune system and also with the inflammatory process that gives rise to the demyelinating lesion. The authors review extensively these various prospects and their immediate implications for multiple sclerosis patients.

Autoantigens↗
PubMed1995

[Stabilization treatments of migraine].

Pathophysiology and principles for diagnosis of migraine, in particular ways to obtain an accurate history between attacks, are presented in the introduction. Identification of migraine triggers is an extremely important part of migraine therapy. Selected pain-relieving drugs should include primarily simple analgesics and antiemetics, while sumatriptan should be reserved mainly for severe migraine attacks. Pharmacotherapy, psychological and physical therapy are all components of a systemic approach to the treatment of migraine. To discontinue overused medication is the first step in the prevention of migraine. A number of available drugs are able to reduce the frequency and severity of migraine attacks. Several considerations should be evaluated before symptomatic and prophylactic therapies are introduced. Our strategies are based on experience collected during the treatment of 82 patients.

Analgesics↗
PubMed1995

[Drug-induced neurological complications in a hospital cohort].

Among the patients admitted to our clinic between 1988-1994, we selected 109 with a neurologic disorder induced by medication. We registered 34 cases of drug-induced headache, 33 of extrapyramidal syndrome, 10 of polyneuropathy, and 5 of myopathy. 12 patients presented neurologic side effects of antiepileptic drugs, and 15 were admitted for epileptic seizures or neuropsychological alterations induced by drugs. The purpose of this article is to review the clinical symptoms of these types of neurologic disorders. It also discusses the drug-induced neurologic complications encountered in general practice. Therapeutic advice, and particularly the choice of psychotropic drugs for epileptic or elderly patients, is proposed.

Anticonvulsants↗