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Atherosclerosis is the main cause of morbidity and mortality in the industrial countries. Although its ethiopatogenesis is not exactly known, it is accepted that the atheromatous plaque progresses in cycles of activity and quiescence. The process is initiated when the endothelium is injured and some of the cells die. Subsequently the necrotic area is covered by platelets and posteriorly new endothelial cells grow on this surface. These new endothelial cells have increased permeability to lipids and lipoproteins, and fatty material is deposited in the subendothelial space. This fatty material is very irritating and produce an inflammatory reaction. As a reaction macrophages migrate to the subendothelial space and phagocytize the fatty material and damaged endothelial cells. The macrophages full of lipids are called the foam cells and they express Fc receptors on the surface. Standard imaging techniques (sonography, doppler, computed tomography,angiography and magnetic nuclear resonance) used in the diagnosis of atherosclerotic disease allows identification of changes in wall thickness, in the turbulence of blood flow or in lumen diameter. Unfortunately all these changes appear once the atheromatous plaque is evolutionated, and they are not useful in the early diagnosis of lesions. In the search for a non invasive technique that allows the diagnostic of the atheromatous plaques in the early stages, different tracers have been used, the most significant being platelets3, 4, 5 and low density lipoproteinsl2, 15. 16. In this study we have used two different tracers the I I lin-platelets and the 99mTc-polyclonal immunoglobulin G (HIG), in patients with cerebro-vascular disease.

We report the case of a patient with multifocal motor neuropathy. Electrophysiological studies showed typical multifocal conduction blocks, but the search for anti-GM1 antibodies was negative. This case provide the heterogeneity of this clinical entity.

We report a series of 46 patients (32 women and 14 men) with limb-girdle syndrome. After reappraisal, another diagnosis was made in 10 of them. Becker's muscular dystrophy was the most frequent cause among men (near 50 p. 100). A Duchenne muscular dystrophy manifesting carrier was discovered among 13 reevaluated women. Among the 36 cases (29 women and 7 men) without any defined etiology, 29 were without any other known familial history. Fifteen of these women had similar clinical findings: incipient weakness in the pelvic girdle and onset of symptoms most often in the forties. In these cases serum creatine kinase activity was normal or slightly elevated, and muscle biopsy showed non-specific patterns. "Late onset muscular dystrophy in females" should be reevaluated.

The main clinical and biological features of 22 cases of neurological forms of Lyme disease are reviewed. Radiculitis (n = 15), cranial nevritis (n = 7), meningitis (n = 5) and encephalitis (n = 4) are often associated. Tick bites were previously noticed in only 40% of cases; erythema chronicum migrans in 73%, "one to six months before the onset of neurological symptoms". Titers of Borrelia Burgdorferi antibodies were always above 1/256. Among 18 patients, DR W2 HLA haplotype was present in 15.

Injection of phospholipase A2 (PLA2) in the rat skin produced a significant rise in oedema, which was inhibited by the simultaneous coinjection of aristolochic acid (100 micrograms), mepacrine (100 micrograms) and p-bromophenacyl bromide (10 micrograms). Indomethacin, nordihydroguaiaretic acid and WEB 2086 were without inhibitory effect on this model, whereas dexamethasone (5 mg/kg, p.o.) and coinjection of chlorpheniramine (20 micrograms) inhibited the oedema formation by more than 60%. Dose-dependent histamine release by rat peritoneal cells was induced by PLA2 and by lysophosphatidylserine and this effect could be antagonized by aristolochic acid and mepacrine. Apomorphine, previously reported to be an antagonist at lysophospholipid receptors, was able to inhibit the histamine release by mast cells as well as the oedema formation in rat skin. Taken all together, these results suggest that lysophospholipids, produced by the action of PLA2, are the mediators for the histamine release in rat peritoneal cells and could play an important role in the early oedema development in rat skin after PLA2 administration.

A 21-year old women with rhizomelic muscular deficit and signs of hypercapnia developed acute respiratory failure. Laboratory tests revealed high creatine kinase activity, and electromyograms showed myogenic patterns with a few myotonic discharges. Biopsy of the quadriceps muscle elicited major vacuolar myopathy with glycogen overload. Acid maltase activity was undetectable in muscular tissue. After 7 months on high-protein diet (1540 calories, 37% proteins) there was no clinical or biochemical improvement. The other published cases of acid maltase deficiency treated with high-protein diet are discussed.

In a family 6 members in 3 generations were affected by centronuclear myopathy (CNM) of autosomal dominant inheritance. The apparent onset was in the early forties and the disease progressed slowly. Limb weakness was predominant. Strabismus was present in 5 cases and calves hypertrophy in 3. Serum creatinine kinase was always within the normal range. In one case myotonic bursts were found at electromyography. In 2 cases brain stem auditory evoked potential studies demonstrated abnormal prolongation of interpeak latencies I-III and favoured subclinical nervous system involvement. Muscular biopsies showed typical features of centronuclear myopathy with 50 to 80% central nuclei. In two cases immunocytochemical labelling of dystrophin showed staining in the sarcoplasm in favour of an arrest in the morphogenesis of developing myofiber. Others families with autosomal dominant CNM in the literature and also some sporadic adult cases had similar clinical features.

A prospective study was conducted to estimate the current magnitude of adherence to short-course tuberculosis treatment, the degree of abandonment, the characteristics of treatment dropouts, and the causes of this abandonment. The study group was made up of tuberculosis patients over the age of 15 who received care at the Western and Southern Health Services of the Metropolitan Region of Santiago, Chile, between 1 October 1987 and 31 January 1988. The percentage abandoning treatment, calculated by the life table method, was 11.5. The profile of patients who dropped out of treatment was as follows: male, under 45 years of age, single, low level of education, no steady work, homeless, and alcoholic. In addition, an opinion survey on the variables associated with abandonment was conducted and it was concluded that the main ones were alcoholism and intolerance to tuberculosis drugs. Awareness of this profile makes it possible to take measures to prevent patients from abandoning treatment, as well as to educate and even hospitalize at the start of treatment those tuberculous patients exhibiting such a profile.

In the necropsy exam of the brains of 13 patients with acquired immune deficiency syndrome (AIDS) cerebrovascular lesions were seen in 5 cases, three of them having AIDS encephalopathy. Hyalinosis was noted in 3 cases, and endarteritis obliterans and fibrinoid necrosis in one case each; both of them had associated perivascular lymphocytic infiltrates. Cerebral infarction was observed in one case, but no hemorrhages were seen. The neuropathological anomalies in four children affected with the AIDS are reported too, being an extensive calcified vasculopathy and changes suggesting active AIDS encephalopathy the main findings.

We report a case of severe subacute autonomic and sensory neuropathy in a 52 year-old man. Cerebrospinal fluid protein was 275 mg/dl. Electrophysiological data were consistent with an axonal sensory neuropathy. Nerve biopsy showed a severe decrease in myelinated fibers, and a less severe loss of unmyelinated fibers. No cause was found and recovery was almost complete over 4 years, with minimal persistent dysautonomia. This case and 4 similar reported cases are compared with pure dysautonomia and with sensorimotor dysautonomic neuropathy. The site of damage is discussed and it is suggested that these cases are axonal forms of inflammatory polyneuropathy.

Two cases of polyneuropathy in patients with hypothyroidism are reported. In both cases, the polyneuropathy involved the lower limbs and was predominantly distal. It was sensorimotor in the first patient and purely sensory in the second one. Electrophysiological findings were consistent with an axonopathy. Symptoms and electrophysiological parameters improved with thyroid therapy. Neuropathy in such cases is probably related to the duration of hypothyroidism.

Thalamic atrophy is rarely primitive. Selective atrophy of the dorso-medial and anterior thalamic nuclei has been reported in a few families. The clinical course is subacute. Symptoms and signs include sleep disorders and intellectual deficit. We report a new family with this uncommon disease and discuss its etiology.

We report a case of choreo-acanthocytosis in a 33 year-old man with mild chorea of the limbs but no involuntary movements of the face, areflexia, epilepsy and personality changes. Acanthocytosis was 10-20% and creatine-phosphokinase levels were raised. Electrophysiological data were consistent with lower motor neurone dysfunction. Muscle biopsy revealed changes typical of neurogenic atrophy. Nerve biopsy showed a severe loss of large myelinated axons. Electron microscopic examination was in favour of primary axonal damage affecting mainly myelinated fibers and only slightly unmyelinated fibers.

Introduccion. La cloroquina es un farmaco ampliamente utilizado en reumatologia y, ocasionalmente, en dermatologia. Desde el punto de vista neurotoxicologico, la cloroquina puede afectar a los nervios perifericos, a los musculos, a la union neuromuscular y al sistema nervioso central. En el presente trabajo se analizan los hallazgos clinicos, neurofisiologicos y anatomopatologicos de dos pacientes con una neuromiopatia inducida por cloroquina, que se manifestaron como una polirradiculoneuropatia. Casos clinicos. Caso 1. Mujer de 75 anos con artritis reumatoide tratada con cloroquina 250 mg/dia durante cuatro anos. Consulto por tetraparesia progresiva de predominio proximal de meses de evolucion con arreflexia. Las pruebas analiticas y la puncion lumbar fueron normales. Electromiograma (EMG): patrones miopaticos proximales y neuropaticos distales. Biopsia muscular: miopatia vacuolar con acumulo de fagolisosomas, lipidos, lipofucsina, cuerpos mielinicos y curvilineos. Caso 2. Mujer de 74 anos con artropatia tratada con cloroquina 250 mg/dia durante nueve meses. Presento una paraparesia proximal progresiva con arreflexia universal. Las pruebas analiticas y la puncion lumbar fueron normales. EMG: polineuropatia mixta sensitivomotora, patron miogeno con descargas de alta frecuencia en psoas iliaco y patron neurogeno en los musculos distales. Biopsia muscular: miopatia vacuolar sugestiva de miopatia por cloroquina. Tras la retirada de la medicacion presentaron una evolucion favorable de la clinica. Conclusion. La cloroquina puede inducir una cuadro clinico sugestivo de polirradiculoneuropatia. Es importante interrogar sobre el antecedente de ingesta del farmaco. En caso positivo tiene interes el estudio electromiografico de los musculos mas proximales para detectar patron miogeno y de los musculos distales para evidenciar patron neurogeno. La biopsia muscular establecera el diagnostico definitivo.

Eleven patients with IgM anti-MAG monoclonal gammopathy of undetermined significance and neuropathy have been treated with high-dose intravenous immunoglobulins (400 mg/kg/day for five days). Eight patients had a subjective improvement and underwent two more courses. In five cases a clear-cut improvement was noted on ataxia and disability scales. Serum IgM levels and nerve conduction velocities remained unchanged.

Resume Les auteurs rapportent le cas d'un patient de 12 ans, presentant une comitialite a crises partielles complexes, evoluant vers un tableau d'epilepsie avec pointes-ondes continues au cours du sommeil. L'introduction du clobazam est suivie chez ce patient d'une amelioration spectaculaire. Les particularites de cette observation sont discutees, ainsi que le role du Clobazam dans la reversibilite des troubles.

Summary Two cases of predominantly motor polyneuropathy attributable to the inhalation of a glue containing n-hexane with methylethylcetone are reported. Electromyographic study shows neurogenic changes with fibrillation potentials at rest in distal muscles and reduced conduction velocities. These findings can be related to the presence of axonal failure and segmental demyelination. Delayed conduction of the brain stem auditory evoked responses suggests an involvement of the central nervous system.

Demyelinating lesions of MS are infiltrated by activated T-lymphocytes and macrophages with secretion of soluble factors. This results in the synthesis of oligoclonal immunoglobulin (IgG) by plasma cells. The activated T-lymphocytes migrate from the peripheral blood to the CNS. This hyperactive state is linked to a selective loss of the suppressor/inducer T-cell subset. Administration of a soluble factor — interferon γ — enhances the immune response by promoting class II antigen expression on macrophages or astrocytes, resulting in a relapse. However, the reason for T-cell activation in peripheral blood is not known, nor is the antigen. Myelin basic protein (MBP) has been considered to be the target since MBP is able to induce chronic relapsing allergic eccephalomyelitis (CRAE) in an animal model of MS. Yet other myelin antigens have succeeded in inducing CRAE in animal models, and anti-MBP antibodies have been found in healthy individuals. The possibility that the hyperimmune state results from a viral infection has not yet been proven. It is known that in Caucasians, a genetic susceptibility factor is linked to class II MHC. Using MRI it has been found that the presence of new plaques was not regularly correlated with relapses, which indicates that MS is an ongoing pathology process. Most drugs used in MS influence the Immune response but have potential toxicity. Monoclonal antibodies offer the opportunity of specific targeting of T-cells and are promising for the future.

Summary In two cases with recurrent palsies, the results of electrophysiological studies led to nerve biopsy showing typical tomacula. The first case was an inherited neuropathy with liability to pressure palsies. The second case was an apparently sporadic painless recurrent brachial neuropathy. Electrophysiological alterations were diffuse and sensory fibres of the median nerve between index and wrist were the most involved. Conduction blocks were observed without palsy in narrow anatomical passageways where nerve compressions are frequent (ulnar nerve at the elbow, peroneal nerve at the fibula). A compression by a neighbouring anatomical structure could make the prognosis worse and justify nerve decompression. The nerves with slowest conduction have the most important risk of palsy and the patients should be given advice to avoid their compression.

In both the cases described, painful ophthalmoplegia was the first indication of infiltration of the cavernous sinus by a lymphosarcoma. The onset of symptoms and the course of the disease were different in the two cases. CT scan which has been the crucial investigation for detecting lymphomas in the cavernous sinus, was normal in the early stages. Symptomatic remission could be obtained with treatment, although the prognosis remained poor.

A blocking factor is present in the serum of patients with multiple sclerosis (MS). It is able to inhibit a cellular cytotoxicity reaction in vitro. This substance has characteristics similar to those of the 'P4' protein described in pregnant women and patients bearing tumors. Its possible contribution to MS pathogenesis is discussed.

Three patients developed a progressive flaccid paraparesis without sensory or sphincter disturbances, following radiotherapy for lymphoma in two cases and carcinoma of testis in one case. The course was progressive with stabilization between two and four years. Electrophysiological study suggested anterior horn cell damage the mechanism of which remains unclear.

A patient with no previous relevant history presented with recurrent cerebral hemorrhages of which he died 4 years 5 months after the onset. On autopsy, an amyloid angiopathy was present and localised to the cerebrovascular system. Hemorrhages were associated with some small infarcts in the subcortical regions. The leptomeninges and the cortical arteries were mostly involved by an infiltration mainly in the adventitia and media. There were no senile plaques in the cortex. The amyloid deposit present in the senile plaques and in the vessel walls of the amyloid angiopathy could be the result of two distinct pathological processes.

Clinical, electromyographic and neuropathological studies were carried out at different stages of evolution of a familial amyloid neuropathy in 6 members of 3 families of French origin. The clinical onset was marked by sensory symptoms and signs in limb extremities, primary manifestations being alterations in pain and thermal sensitivity. This was followed by motor and amyotrophic disorders predominant in the lower limbs. Autonomic nervous system disorders were frequent later. Early electromyographic signs were diminished amplitude and increased duration of sensory potentials. Progression of the disease is shown by the onset of signs resulting from severe axonomyelinic lesions. Neuromuscular biopsy demonstrated the presence of amyloid deposits in the endoneurium in 5 of the 6 cases. Transmission appeared to be dominant autosomal. These cases pertain to group I of the amyloid neuropathies. The axonal lesions marking the onset of the affection could be secondary to biochemical alterations in prealbumin, responsible for amyloid formation. This hypothesis affords a basis for plasmapheresis which has been used in 3 patients.

In two cases with recurrent palsies, the results of electrophysiological studies led to nerve biopsy showing typical tomacula. The first case was an inherited neuropathy with liability to pressure palsies. The second case was an apparently sporadic painless recurrent brachial neuropathy. Electrophysiological alterations were diffuse and sensory fibres of the median nerve between index and wrist were the most involved. Conduction blocks were observed without palsy in narrow anatomical passageways where nerve compressions are frequent (ulnar nerve at the elbow, peroneal nerve at the fibula). A compression by a neighbouring anatomical structure could make the prognosis worse and justify nerve decompression. The nerves with slowest conduction have the most important risk of palsy and the patients should be given advice to avoid their compression.

The authors describe the case of a 12 years old boy suffering from an epilepsy with complex partial seizures evolving to a syndrome of epilepsy with continuous spikes-waves during sleep. A dramatic improvement follows clobazam introduction in the treatment. The originality of this case report and the possible mechanism of action of clobazam are discussed.

A patients is reported with a progressive neurological disease which started at the age of 19 years and included spasmodic paraplegia, urinary disorders, impotence and neuropathic signs. The neurological syndrome was associated with adrenal failure. The disease led to death within 5 years. In this characteristic picture of adrenomyeloneuropathy, biopsy of peripheral nerve disclosed lamellar inclusions in the Schwann cell cytoplasm. Such structures, which have already been observed in the sural nerve of some adrenoleukodystrophy cases reported previously, are described for the first time in a case of adrenomyeloneuropathy.

The bifurcated needle vaccination method used successfully against smallpox is a relatively simple technique that can be administered by personnel with only basic preparation and a minimum of training. This fact suggests that it could prove useful in campaigns of BCG vaccination against tuberculosis--especially for vaccination of newborns. With this end in mind, two studies were carried out in Santiago, Chile, to examine previously reported difficulties with the method and to assess its potential as an alternative to the best current method, that of intradermal inoculation. These studies indicated that previously reported variations in the results achieved by different vaccinators could be markedly reduced. However, they also found that the bifurcated needle method did not cause a sufficiently large dose of vaccine to enter the subject--even when fifteen needle strokes and a highly concentrated vaccine (160 mg per ml) were employed. It was thus concluded that although modification of the needle or other changes might ultimately yield satisfactory results, the bifurcated needle technique cannot yet be recommended for BCG vaccination of newborns.

Muscle hypertrophy in neurogenic disorders is an uncommon phenomenon which has been reported in various conditions: hereditary or acquired anterior horn cell diseases, essentially S1 radicular compressions, and polyneuritis generally of a demyelinating type. We report two cases of denervating disease with muscle hypertrophy. The first was an S1 radiculopathy, and the second a compression with ischemia of the spinal cord by herniation of the D11-D12 disc, and showing partial improvement after surgery. In both cases, electrophysiological examination of the hypertrophic muscles revealed abnormal activities identified as complex repetitive discharges. In the second case they were associated with a syndrome of continuous motor unit activity. Such activities are rare in peripheral nerve involvement. However they have quite often been recorded in cases of neurogenic muscle hypertrophy and may therefore be partly responsible for the development of the hypertrophy.

Four patients with idiopathic livedo reticularis and multiple cerebral ischemic events leading to a severe dementia are reported. Imaging (CT, MRI, angiograms) and pathological features (in one case) are presented. Similar clinical features have been described previously in Divry-van Bogaert's disease and Sneddon's syndrome. Data from our cases and a review of the literature did not find distinctive characteristic between these entities. Thus, for practical purposes, considering them as one group appears at present warranted.

A chronic demyelinating neuropathy with "benign" IgM gammopathy was followed for 6 years in a 63-year-old man. The clinical, biologic, and EMG aspects were similar to those already reported, but a lymphoplasmocytic infiltrate in the nerve connective tissue of this patient has only rarely been observed in benign IgM gammopathy. The paraprotein was not evident in the serum until 5 years after symptoms of the neuropathy started.

La breve postfazione colloca i casi di Ceuta e Melilla, a cui il libro è dedicato, all'interno del tempo lungo dell'espansione coloniale spagnola ed europea e all'interno del tempo più breve delle trasformazioni del regime di confine a partire dalla nascita dell'Unione europea.