Few insights on the post-COVID-19 problem.

No abstract Keywords.

[Neurological catastrophes due to medical errors]. / Catástrofes neurológicas por desconocimiento y retraso diagnóstico.

In 1999, the American Institute of Medicine reported an enormous rate of medical errors, representing the fifth cause of death. In Spain, there is no reliable information on the number and nature of medical adverse events, but the situation is probably similar to that described in the USA in 1999, if not higher. Diagnostic errors account for more than half of neurological adverse events and these errors can be catastrophic if the natural progression of the neurological disorder causes severe sequels or even death when the patient is left untreated. To improve patient safety, research must be undertaken to determine how these errors are produced and to develop strategies to prevent inappropriate conduct. Among many other elements, it is important to create teamwork, improve neurological knowledge among general practitioners and residents, to design clinical practice guidelines aimed at patient safety, and to promote policies that reward the absence of errors. In general, medical errors are neither exclusively due to lack of experience nor to insufficient medical knowledge, but rather to faulty organization of medical care. Therefore, it is preferable to monitor healthcare organization rather than to blame the individual supposedly responsible for the error.

[Advanced dementia in Alzheimer's disease and memantine]. / Demencia avanzada de la enfermedad de Alzheimer y memantina.

INTRODUCTION: Alzheimer's disease (AD) causes dementia with a progressive course. Until now, research has been aimed mainly at studying its early, pre-dementia and mild or mild to moderate dementia phases in an attempt to find treatments with which to cure it or at least halt its progression. It is true that the latest cognitive therapies are effective up to a point, but the fact is that many patients reach the state of advanced AD, which gives rise to a number of cognitive and behavioural disorders that entail all kinds of problems on a personal, familial and community health level. AIMS: This study reviews the progress of AD, the length of its stages, the speed at which the disease courses and patients' survival. Advanced AD is defined, its clinical features and the functional disability it causes are described, and we analyse the tests and scales that must be used to measure how the process is progressing and the effectiveness of the distinct forms of treatment used in the later phase of AD, which are different to those employed in the early stages. All this allows us to analyse the results of clinical trials carried out with memantine, an NMDA (N-methyl D-aspartate) receptor antagonist. We review its pharmacological characteristics and its use in everyday practice. Lastly, we refer to the classical symptomatic treatments that are usually employed to control the frequent and intense behavioural disorders produced in the advanced phase of AD. CONCLUSIONS: The later stage of AD requires our attention because many patients reach and remain for a long time in this phase, which leads to considerable personal and social disorders. Several scales and tests have been adapted to these later phases of AD, enabling the clinician to evaluate the patient, monitor the progress of the disease and determine the effectiveness of different treatments. Memantine, the most recent drug approved for use with this disease, has proved to be effective in the treatment of patients with advanced AD. This pharmaceutical has been added to the list of well-known classical medicines, such as neuroleptic drugs, antidepressants, anxiolytic agents and others, which can be used to diminish the behavioural disorders in these patients and improve their quality of life, as well as that of their caregivers.

Progressive bulbar paralysis associated with neural deafness. A nosological entity.

A complete autopsy verification of progressive bulbar palsy associated with neural deafness was performed. Hearing loss and speech difficulties developed in a five-year-old girl. When she was 24 years old, clinical examination demonstrated deafness and bulbopontine paralysis together with retinitis pigmentosa, peripheral amyotrophies, pyramidal signs, and ataxia. The patients died at 27 years and the autopsy disclosed degenerative changes characterized by simple atrophy and loss of neurons accompanied by gliosis and loss of myelinated fibers. The structures principally affected were the anterior horns and the motor nuclei of the brain stem together with the eighth cranial nerve nuclei. Loss of myelinated fibers was found in the spinocerebellar and pyramidal tracts and in the fasciculus gracilis. Our study suggests that progressive bulbar paralysis with neural deafness should be considered as a nosological entity.

Cluster tic syndrome.

Cluster tic syndrome (CTS) is a disorder characterized by three types of pain attacks. One resembles trigeminal neuralgia, the second resembles cluster headache, and the third is mixed, starting as a neuralgic pain immediately followed by a homolateral headache accompanied by autonomic signs. This type of attack is pathognomonic for CTS. The three pain types can all be provoked. CTS appears between ages 20 and 70 and may be either episodic or chronic; medical treatment is poor.

Anti-Ri antibodies associated with opsoclonus and progressive encephalomyelitis with rigidity.

A 57-year-old woman without a known neoplasia developed opsoclonus, myoclonus, and ataxia. Positive anti-Ri antibodies were present in both serum and CSF. The patient also had progressive encephalomyelitis with rigidity, an association not previously described.

Brachial diplegia in central pontine myelinolysis.

A patient developed weakness in the upper limbs, eventually causing brachial diplegia with only slight paresis of the legs after rapid correction of severe hyponatraemia. Pseudobulbar palsy, mental confusion and urinary incontinence were also present. CT scan showed a zone of lucency in the pons. Clinical recovery occurred and the zone of lucency had disappeared 12 months after the appearance of the neurological signs.

Hereditary amyotrophic lateral sclerosis.

A Spanish family transmits, as an autosomal dominant trait, a form of amyotrophic lateral sclerosis characterized by an unusually prolonged evolution of the disease in all affected members. Precocity and persistence of muscle cramps, presence of unilateral proximal segmental myoclonus and early abolition of ankle jerks are other clinical features conspicuous in this family. This type of hereditary ALS of non-chamorro origin and prolonged evolution is rare.

Hereditary distal myopathy with onset in early infancy. Observation of a family.

The study of a family affected with hereditary distal myopathy with onset in early infancy is presented. Complete neurological examination was necessary in several members of the two last generations to discover the existence of the abnormalities of which they were unaware. The propositus was the most affected member of the family iwth distal paresis of the upper and lower extremities and selective paresis of the deltoid muscles. In addition he had kyphoscoliosis, talipes valgus and limitation of mobility of several joints. The onset of the disease was estimated as before the age of 2 when the child started walking. There was no progression of the disease. Clinical examination suggested a myopathic origin of the condition. A sural nerve biopsy was normal. Light-microscopy histochemical studies disclosed a predominance of type I fibres which were at the same time hypotrophic. Subsarcolemmal deposits of mitochondria were present although they were scanty and of normal ultrastructural appearance. In view of the morphological presentation it is postulated that this disease should be classified within the groups of myopathies accompanied by disproportion of fibres and selective atrophy of type I fibres.

Glossopharyngeal and vagal neuralgia secondary to vascular compression of the nerves.

Glossopharyngeal and vagal neuralgia secondary to neurovascular compression of the 9th and 10th cranial nerves is reported. Microsurgical decompression of the nerves provided relief from the symptoms.

[Posterior cortical atrophy]. / Atrofia cortical posterior.

INTRODUCTION: Posterior cortical atrophy (PCA) is a dementing syndrome characterized by an early alteration of higher visual functions. Patients usually develop a perceptive visual agnosia related to Balint syndrome. DEVELOPMENT: Verification showed Alzheimer s disease (AD) in most PCA observations (13/14 cases), and it can be concluded that there is a posterior variant of AD with prominent visual symptomatology. However, most PCA cases have not been verified and the nature of the disorder remains unknown in these observations. An early and severe unilateral occipital horn dilatation was found in one out of every six cases with non verified PCA. To the best of our knowledge, this image has not been described in AD and these observations could be of different nature. On the other hand, some non verified PCA cases have occasionally manifested as an associative visual agnosia. The nature of this type of associative disorder is unknown, but it could be similar to the nature of semantic dementia, a non Alzheimer s syndrome related to unspecific lesions of temporal lobes.

[Epilepsy and migraine]. / Epilepsia y jaqueca.

Migraine and epilepsy are two clearly different syndromes. All they have in common is that both cause paroxystic neurological phenomena. However, the frequency of epilepsy in patients with migraine, and migraine in those with epilepsy seems to be higher than one would expect. This suggests there may be comorbidity in both conditions. Analysis of this comorbidity is very important since it may give clues as to the physio-pathology and aetiology of certain disorders. This paper analyses the existence of migraine-epilepsy comorbidity and the levels at which it occurs. This coexistence may be due to the episode of one, for example the aura of a migrainous attack, triggering off the other condition, that is an epileptic crisis. So, it may be that the 'migrainous illness' causes the 'epileptic illness' or the other way around. Their coexistence may be due to a risk factor which is common to both, since it has caused a cerebral lesion which is the cause of both disorders. Finally, a risk factor may have a direct effect, without requiring the intervention of an intermediate cerebral lesion to cause both migraine and epilepsy. This last possibility is particularly attractive to explain the comorbidity of migraine with an aura and genetically determined epilepsy.

[Alzheimer's disease and women]. / Enfermedad de Alzheimer y mujer.

INTRODUCTION AND DEVELOPMENT: This work reviews the relation between Alzheimer s disease (AD) and women, a very interesting issue both for its socio economic, and etiopathogenic and therapeutic aspects. Much of the prevalent research conducted in this field shows that a higher proportion of suffers from this disease are women, and in the work on incidence there is at least a tendency toward the same conclusion, especially at a very advanced age. In fact, the risk of suffering from AD is greater among women and most of the patients we attend are females, which is to a large extent associated with the fact that women live longer. However, it is possible that there are other biological factors involved and for this reason the action of estrogens on the brain and the consequences of women s being deprived of them during menopause is of special interest. CONCLUSIONS: Different studies have shown that the administration of hormone replacement therapy (HRT) lowers the risk of suffering from this disease, although design defects make it necessary to wait for the conclusions from other research work currently being conducted. There are also data that supports the idea that HRT can be beneficial in AD if it is administered in suitable doses. Obviously gender can influence or modulate other risk factors (RF). Genetic factors are not easily modified and for this reason research is currently aimed at factors in which a strong environmental component is involved. Another very controversial possible RF is lack of schooling, but some data support the notion that its influence can be especially harmful among females. This is a very important hypothesis because women make up the greater part of the illiterate population in Spain. Finally, women are also prevalent among caregivers and, therefore, suffer AD from both angles: they must care and be cared for. The reaction to this situation seems to be gender specific, which means that women in particular suffer the consequences of the lack of reciprocity brought about by AD something that does not happen in other equally devastating chronic processes, but which affect the physical sphere.

[Pregnancy and benign intracranial hypertension]. / Embarazo e hipertensión intracraneal benigna.

Several papers have suggested that pregnancy is one of the etiopathogenic factors of benign intracranial hypertension (BIH). The therapeutic attitude to be taken as regards new pregnancies in women previously afflicted with BIH during pregnancy is still on discussion. This paper is based on a study of 100 BIH cases. The results support the idea that cases, but the obesity involved. The coexistence of BIH and pregnancy does not increase the risk of relapse, does not mean a worse prognosis of BIH nor does it appear to have a negative effect on the child. Any woman who has previously developed BIH during pregnancy should not be advised against future pregnancies. In the case of a new pregnancy, a very close control should be carried out in order to avoid an excessive increase in weight.

[Recurrence of benign intracranial hypertension]. / Recidivas en la hipertensión intracraneal benigna.

The recurrence of benign intracranial hypertension (BIH) in 100 patients was analysed after a long-term follow-up. A recurrence appeared in 20% of the cases in this series. This being more frequent, with statistical significance, in females aged between 20-40 years. Obesity was the more frequent etiopathogenic factor involved in the onset of relapses. Other factors were pregnancy, steroid therapy, levothyroxine and obstruction of the cerebrospinal fluid derivation system. Relapses did not produce loss of sight. We insist on the elimination of developing factors in order to prevent the recurrence.