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Biomedical subjects

J M García-Moreno

Publications and source records attributed to J M García-Moreno.

At least 19 recordsLinked to original sources
PubMed2002

[Lhermitte's sign].

Besides Babinski's, Lhermitte's sign is likely the eponym mostly used in the neurological literature. We review here the history of this eponym as well as recent advances on its pathophysiology and treatment. Lhermitte's phenomenon is, on one hand, a symptom as it is spontaneously explained by patients, and, on the other hand, a sign as it may be triggered by flexion of the nape. Initially described after head and cervical spine trauma, firstly by Marie and then by Babinski, it was Jean Lhermitte who recognized on it an etiological specificity, namely, a demyelinating sign of cervical spinal posterior cords. He also made a pathophysiological interpretation of the phenomenon, namely, a stretching of posterior cords during flexion of the neck. All authors agree that this phenomenon is more common in multiple sclerosis, although it has been descibed in many other conditions. The history of how this sign was hatched, whose pathophysiology remains a mystery, is fascinating. And it is fascinating the fact that patients compare the phenomenon with a current, especially if we bear in mind that few people may have suffered an electrocution, mainly in that time when just a few ones could actually enjoy domestic electricity.

Cervical Vertebrae↗
PubMed1999

[Evolution and adverse effects in patients with remittent-recurrent multiple sclerosis treated with interferon beta 1b. The influence of patient's weight and height].

BACKGROUND: The aim of the present study was to determine whether the adverse effects (AE) of interferon beta 1b (IFNb-1b) are related to patient weight, height and body surface area. Moreover, whether the basal incapacity and/or some of these AE may be related to the evolutive prognosis were also studied. METHODS: Twenty-nine females and 14 males with remittent-recurrent multiple sclerosis treated with IFNb-1b were studied. The clinical data and the AE, were compared with the number of outbreaks and the progression of incapacity over the first year of treatment in patients with high weight, height and body surface area with respect to those under the mean. RESULTS: Although the presence of fever was similar in the two groups during the first month (8/19 in the high weight patients and 11/24 in the low weight group), in the following months, overall, the low weight patients continued to present fever. Fever during the first month was associated with a lower number of outbreaks at one year of evolution (0.04 +/- 0.1, n = 19 vs 0.30 +/- 0.5, n = 24; p = 0.04). Furthermore, the patients with lesser incapacity presented a better evolution than those with more incapacity, particularly at 2 years (0.21 +/- 1.3 vs 1.36 +/- 0.9; p = 0.05). CONCLUSIONS: During the first month of treatment fever is very frequent in all the patients regardless of weight and fever was correlated with a lower number of outbreaks at one year of evolution. From the third month fever was less frequent in the lower weight patients group.

Adjuvants, Immunologic↗
PubMed1998

[Familial cerebral cavernomatosis associated with cutaneous angiomas].

UNLABELLED: OBJECTIVE, PATIENTS AND METHOD: We present a genealogical study of a person with familial cerebral cavernomatosis (CCF) discovered following study of a man who presented with status epilepticus at the age of 35 years. This had also affected another three members of the family consisting of a total of 43 persons, studied over six generations. RESULTS: In the family members studied we did not find cavernomas at other sites but there were dermatological lesions whose relation to cerebral cavernomas (CC) is discussed. CCs are the second commonest type of cerebral vascular malformations. Their major clinical features are epilepsy and cerebral haemorrhage. There are two forms of CC: a sporadic form with single lesions and a familial form with multiple lesions. Although the condition has been recognized since 1854, very little is known about it. Recently a gene causing the familial form has been found on chromasome 7. CONCLUSIONS: We discuss the clinical findings in this family with relation to descriptions in the literature. We emphasize the importance of MR in diagnosis of the condition and for detection of asumptomatic carriers. We consider a possible relationship between these findings and genes which have been mapped, and in view of recent genetic data, the hypothesis of a possible Spanish ancestor with the condition.

Adolescent↗
PubMed1998

[Changes in lymphocyte subsets s and treatment with beta interferon in active multiple sclerosis].

INTRODUCTION: We report that interferon beta decreases CD8 T cells percentage and increases CD4/CD8 cell's rate in vivo in Multiple Sclerosis (MS) patients. PATIENTS AND METHODS: We studied 40 patients (22 women and 18 men) with clinically definite active MS who received IFN beta. Twenty-six were treated with nIFN (9 MU/week) and 14 with rIFN (28 MU/week). All patients except two with secondary progressive forms presented relapsing remitting courses. Mean age and mean age at onset were 36.5 +/- 9 and 27.8 +/- 7 years respectively. Mean EDSS score was 2.96 +/- 1.8. Patients were reviewed at four weeks and every eight weeks and periodical studies of immunity were performed. T cell subpopulations (CD3, CD4, CD8 and NK) were studied byflow cytometry. RESULTS: The evolution of CD8 T cell percentage showed a statistically significant decrease in all blood samples after 20 weeks of treatment with rIFN (24.3 +/- 8 vs 34.7 +/- 5 in the control group) and after 36 weeks for nIFN beta group (25.7 +/- 6 vs 33.0 +/- 4 in the control group). No changes were detected in CD4 T cell subset. The evolution of CD4/CD8 T cell rate showed an increase over the cut-off (2.200) in all blood samples after 20 weeks of treatment with rIFN (2.302 +/- 1.12, 2.332 +/- 0.99 and 2.488 +/- 1.61 for 20, 28 and 36 weeks respectively) and after 52 weeks for nIFN beta group (2.128 +/- 1.07, 2.346 +/- 1.09 and 3.168 +/- 3.87 for 52, 60 and 68 weeks respectively). CONCLUSIONS: Both nIFN and rIFN beta are able in vivo to decrease CD8 percentage of T cells and increase CD4/CD8+ T cell rate. The increase in the rate is produced earlier in the rIFN treated group.

Adult↗
PubMed1997

[Myelographic diagnosis of an epidural arterio-venous fistula].

INTRODUCTION: Epidural arterio-venous fistulae are a little known clinical condition. They are probably commoner than is thought, since diagnosis and angiographic demonstration are difficult. CLINICAL CASE: We present the case of a 49 year old man with the clinical features of chronic myelo-radiculopathy with episodes of intermittent medullary claudication. This shows the use of myelography, which in certain cases allows clear detection of vascular structures which might otherwise pass unnoticed. CONCLUSIONS: One should think of this condition when compatible features are found and there is no other diagnosis, since if confirmed by myelography and/or magnetic resonance prior to angiographic study, it may benefit from embolization, which is an effective treatment for this.

Arteriovenous Fistula↗
PubMed1997

[Neuroborreliosis in a patient with progressive supranuclear paralysis. An association or the cause?].

INTRODUCTION: Many different neurological conditions may be seen in the later stages of Lyme's Disease, such as blindness, epileptic crises, CVA, extrapyramidal disorders, amyotrophic lateral sclerosis, and dementia may be yet another form of presentation of chronic infection due to Borrelia burgdorferi (Bb). Progressive Supranuclear Paralysis (PSP), a disorder of unknown aetiology, considered to be the commonest cause of Parkinsonism-plus, one of the symptoms of which is dementia, has never been mentioned in this type of differential diagnosis. CLINICAL CASE: We present the case of a 78 year old man with sub-acute mental deterioration, Bb positive serology in both plasma and CSF, and with clinical and epidemiological features compatible with Lyme's Disease. Complementary tests were negative. The syndrome corresponded to Lyme's Disease and improved after treatment with ceftriaxona. CONCLUSIONS: We consider aspects of the aetiology of PSP which are still not clear. In our patient, the aetiology seemed to be Bb infection, according to the criteria of the original description of the disease and in view of the neuropathological findings which have shown Bb in the substancia nigra of the mid-brain and the existence of an animal model in which Bb shows a particular tendency to colonize infratentorial structures.

Aged↗
PubMedDate not supplied

[Clinical features of familial multiple sclerosis in Spain].

INTRODUCTION: Multiple sclerosis (MS) is a demyelinating and inflammatory disease of the central nervous system (CNS) with a clear proved genetic susceptibility. The real frequency and clinical features of familial MS is although not well established in Spain. OBJECTIVE: We studied the clinical and CSF features of familial MS (FMS) patients in comparison with the rest of our patients. PATIENTS AND METHODS: We reviewed 308 definite MS patients looking for patients with other familial members with MS. We analyzed clinical characteristics (age, age at onset, sex, evolution time, evolution course, symptoms at onset, disability measured by EDSS scale) and IgG synthesis measured by Tibbling-Link index. RESULTS: We found 23 patients (10 men and 13 women) in 18 independent families with at least other family member diagnosed of definite MS (7.47% of our total MS population). Age and age at onset were no different from non FMS cases. The clinical course was relapsing-remitting in 21 out of 23 FMS cases and secondary progressive in two. No primary progressive cases were found among FMS. At onset the symptom most frequently found in FMS was optic neuritis. Mean EDSS score was lower in FMS cases in comparison with the rest of cases. Link index was increase in 93.7% of patients with FMS. CONCLUSION: In comparison with non familiar forms FMS patients in Spain, present more often remitting courses, are less disabled, optical neuritis is more frequently seen as onset symptom and IgG synthesis is more often increased.

Adult↗
PubMedDate not supplied

[Parkinson disease of juvenile onset with systemic lupus erythematosus in a pre-symptomatic stage].

INTRODUCTION: Parkinson's disease of juvenile onset is known to be rarely seen in clinical neurology. Occasionally it may be secondary to other pathological processes which have to be excluded by investigation of such cases, since the clinical, therapeutic and prognostic significance varies considerably depending on whether the condition is idiopathic or secondary. CLINICAL CASE: We present the case of a patient whose illness started when she was 16 years old. She complained of rigidity, tremor and akinesia. Her condition was studied to find whether the syndrome was secondary. Thus, on four occasions, at annual visits during a period of five years, immunological changes suggestive of systemic lupus erythematous were observed. Neuroimaging, magnetic resonance and computerized axial tomography studies were completely normal on all four occasions over the five years during which the patient had annual follow-up reviews. CONCLUSION: We considered whether the rigidity-akinesia-tremor syndrome might have been secondary to systemic lupus erythematous, due to a probable pathological focus of cerebral vasculitis, in this clinical case which we report.

Adolescent↗
PubMedDate not supplied

[Intrathecal perfusion pump with baclofen in generalized dystonia].

OBJECTIVES: We show the effectiveness of treatment with continuous intrathecal baclofen infusion in a case of hereditary generalized dystonia refractory to anticholinergics, tetrabenazine, pimozide, L-dopa, benzodiazepines and thalamotomy. CLINICAL CASE: A 26 years old female patient, when she was 11 years old began with torsion dystonia in her left feet, that progressively worsened to involve her entire body. She had painful spasms. She had four brothers, three of them with dystonia and one healthy. Her uncle grandfather had similar symptoms. Complementary explorations to reject secondary origin was negatives. She was treated with high and progressive dosages of anticholinergics, pimozide, tetrabenazine, benzodiazepines, L-dopa and thalamotomy without improvement. Underwent intrathecal baclofen test dosing, we used 25, 50, 100 micrograms/day, the last one with improvement during 10 hours. A pump was inserted with an initial dose of 220 micrograms/day. After pump insertion, baclofen dosage was gradually increased to 450 micrograms/day. She had a great improvement in right part of her body and less in her left body. Painful spasms had disappeared. CONCLUSION: We propose continuous baclofen intrathecal infusion pump for patients with severe torsion dystonia that not response to ordinal treatment.

Adult↗
PubMedDate not supplied

[Neuropsychiatric disorders in multiple sclerosis].

OBJECTIVE: Neuropsychological investigations over the past 20 years have shown the high prevalence of cognitive dysfunction in multiple sclerosis, although there are still large gaps. In this article we review current concepts regarding the different types of neuropsychiatric disorders, their physiopathology, diagnosis and treatment. DEVELOPMENT: We present a classification of the most important types of cognitive and psychiatric alterations in multiple sclerosis, and their physiopathology. We review the methods used in diagnosis, follow up and assessment of these patients. Finally, we analyze the current treatment available for each of these disorders. CONCLUSIONS: Although considerable advances have been made in understanding the neuropsychological disorders occurring in multiple sclerosis, there are still many questions to be answered. There is a need for a unified, agreed, validated battery of neuropsychological tests for the study of specific cognitive deficits in multiple sclerosis so that results may be compared. There is also a lack of biological markers which would help in diagnosis and prognosis. Finally the design and carrying out of clinical trials in the search for specific, effective drugs for the treatment of neuroconductual disorders of multiple sclerosis is necessary.

Adjuvants, Immunologic↗
PubMedDate not supplied

[Hypothyroidism concealed by Parkinson's disease].

AIMS: Although it is commonly recognised that diseases of the thyroids can simulate extrapyramidal disorders, a review of the causes of Parkinsonism in the neurology literature shows that they are not usually mentioned or, if so, only very briefly. The development of hypothyroidism in a patient with Parkinson s disease can go undetected, since the course of both diseases can involve similar clinical features. Generally speaking there is always an insistence on the need to conduct a thyroidal hormone study in any patient with symptoms of Parkinson, but no emphasis is put on the need to continue to rule out dysthyroidism throughout the natural course of the disease, in spite of the fact that the concurrence of both pathological conditions can be high and that, in the same way hypothyroidism can simulate Parkinson s disease, the latter can also conceal hypothyroidism. CASE REPORT: We report the case of a female patient who had been suffering from Parkinson s disease for 17 years and started to present on off fluctuations that did not respond to therapy. Hypothyroidism was observed and the hormone replacement therapy used to resolve the problem allowed the Parkinsonian fluctuations to be controlled. CONCLUSIONS: We believe that it is very wise to suspect hypothyroidism in patients known to be suffering from Parkinson s disease, and especially so in cases where the clinical condition worsens and symptoms no longer respond properly to antiparkinsonian treatment. These observations stress the possible role played by thyroid hormones in dopaminergic metabolism and vice versa.

Aged↗
PubMedDate not supplied

[Pseudotumour cerebri in a patient with Lyme disease and hypothyroidism].

INTRODUCTION: Lyme disease is a complex infectious disease affecting multiple organic systems. The most frequent complications are of a dermatological, rheumatological, ophthalmological, cardiac and neurological nature. Among the neurological complications, pseudotumour cerebri is considered to be a rare manifestation of the disease. Hypothyroidism, however, is known to be only infrequently associated to pseudotumour cerebri. Paparone recently reported a case in which Lyme disease and primary hypothyroidism were concurrent, and Becker and Trock described the case of three patients with concurrent thyrotoxicosis and Lyme disease. CASE REPORT: We present the case of a 19 year old female with pseudotumour cerebri and hypothyroidism secondary to autoimmune thyroiditis, which improved after treating her for concomitant Lyme. CONCLUSIONS: We suggest there is a common aetiopathogenic connection between the three processes through autoimmune thyroiditis triggered by Borrelia burgdorferi. Thus, in this patient, the pseudotumour cerebri could be due to either a direct mechanism, triggered by B. burgdorferi, or indirectly, through hypothyroidism secondary to thyroiditis induced by B. burgdorferi, or perhaps to both mechanisms. It might be wise to consider infection by B. burgdorferi in patients with pseudotumour cerebri or thyroiditis coming from areas in which Lyme disease is endemic

Adult↗
PubMedDate not supplied

[The efficiency and cost-utility ratio of interferon beta in the treatment of multiple sclerosis in Andalusia].

INTRODUCTION: The availability of the interferon beta in its three forms at the moment available in our country and of glatiramer acetate has marked a point of flexion in the natural history of multiple sclerosis (MS), but the high cost of these treatments cause that its use is questioned. In this work we have studied the effectiveness and efficiency of the processing with interferon beta, and the cost-utility of these treatments in MS in Spain has been also analyzed. PATIENTS AND METHODS: For this work we studied 102 patients affected of RR MS, treated with the three interferons beta which we have available in our country. We used as control 330 patients who had participated in the pivotal clinical interferon trials with both interferon beta 1a. In these patients in addition to effectiveness data, we have studied the disability measured as area below curve and the quality of life (AVACs). We also calculated the economical costs, considering the relation cost-utility in our country. RESULTS: Besides to confirm the data of effectiveness of three interferons, in this study a saving of 23 days/year is demonstrated what corresponds to 0.063 AVACs. The additional cost of interferons is greater than the avoided cost until the fifth year of treatment in which the tendency is reversed in favor of the group of treated patients, if we assume that the same effectiveness that we found in the first years is maintained in the long term. CONCLUSION: The use of the treatment with interferon beta is justified by its effectiveness, efficacy and efficiency. The additional cost of the treatment will be compensated in the long term if the effectiveness of the interferon beta is maintained.

Adult↗