[Hypokalemic paralysis disclosing vipoma].
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Aged↗
Biomedical subjects
X Ferrer
Publications and source records attributed to X Ferrer.
[Sensory neuropathy and subacute dysautonomia. Clinical and pathological study].
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.
Autonomic Nervous System Diseases↗
[Compliance with tuberculosis treatment in adults in Santiago, Chile].
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.
Adolescent↗
The influence of Calmette-Guérin bacillus immunization on the booster effect of tuberculin testing in healthy young adults.
The booster or enhancement effect of repeated tuberculin skin testing in Calmette-Guérin bacillus (BCG)-vaccinated young adults was studied in 208 first-year medical, nursing, and medical technology students in Santiago, Chile, where BCG vaccine is usually administered at birth and at 6 and 14 yr of age. Thirty-three students had no BCG scar, 62 had one scar, 71 had two scars, and 42 had three scars. The mean age for each group was 19 yr. All students were healthy and had no known exposure to tuberculosis or history of tuberculosis or other mycobacterioses. The size in millimeters of induration of the first tuberculin reaction (PPD1) was clearly correlated with the number of BCG scars: 2.3 +/- 4.6 for no scars; 6.7 +/- 6.7 for one scar; 10.9 +/- 5.9 for two scars, and 13.2 +/- 5.3 for three scars. The second tuberculin reaction (PPD2), performed 2 wk later on the contralateral forearm, showed a marked increase in reactivity. The increase in reaction size was most evident in students who had BCG scars but who were initially PPD negative (less than 10 mm). Smaller increases were observed in students without BCG scars, and also in those who had BCG scars but who were initially tuberculin positive (greater than or equal to 10 mm). The persistence of the booster effect was evaluated by performing PPD3 1 yr later. PPD1-negative students with BCG scars maintained the increased level of reactivity to PPD2 after 1 yr. An immunizing effect of tuberculin testing was suggested in 11 nonimmunized students who were initially PPD negative.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
[Hypothyroid neuropathy].
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.
Aged↗
[Subacute familial thalamic atrophy. Memory disorders and complete insomnia].
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.
Atrophy↗
[Choreo-acanthocytosis].
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.
Acanthocytes↗
Extensive cerebral calcification and retinal changes in pseudohypoparathyroidism.
The case of a 41-year-old woman with cerebral calcification of a rather unusual extent is reported. This condition was associated with mental deficiency, pseudohypoparathyroidism and Albright's hereditary osteodystrophy. Four years later hypothyroidism was diagnosed. Visual impairment and electroretinogram abnormality suggested a retinopathy involving mostly rods. Despite their rarity, pseudohypoparathyroidism and Albright's hereditary osteodystrophy are of major interest, since they represent the only human disease states in which G protein function has been found to be disrupted. The overall clinical picture was strongly suggestive of a genetic deficiency of a guanine nucleotide-binding protein, termed Gs. The putative involvement of another G protein, contained in rods and cones, transducin, in the pathogenesis of the retinopathy is discussed.
Adult↗
Multiple sclerosis: an overview.
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 gamma--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 encephalomyelitis (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.
Animals↗
Jaw closing spasm--a form of focal dystonia? An electrophysiological study.
The case of a 36 year old man suffering from unilateral right jaw closing spasms over two years is reported. Permanent spasm with trismus severely impeding mouth-opening was combined with paroxysms triggered by various sensory stimuli. The diagnosis of temporo-mandibular joint syndrome was considered but treatment failed to improve the symptoms. Neurological investigation two years after onset of the spasms showed by electrophysiological studies excessive co-contraction of the antagonistic jaw-closers, mainly the right masseter during attempts at jaw opening and absence of the silent period in the right masseter and anterior temporalis following jaw tap and trigeminal exteroceptive stimulation. Jaw dystonia was therefore considered and Botulinum A toxin was injected into the right masseter and temporalis which dramatically improved the patient's condition.
Adult↗
Chronic relapsing idiopathic polyneuropathy with primary axonal lesions.
Idiopathic polyradiculoneuropathy with primary axonal lesions is rarely encountered. Two cases are reported with a chronic relapsing course and a fatal outcome. Neuropathological examination of biopsied peripheral nerve in the two patients and in a necropsy case showed loss of myelinated fibres, but neither active demyelination nor inflammatory cells were observed. Acute and chronic relapsing axonal polyradiculoneuropathies appear to be two clinical forms of a peculiar entity different from GBS.
Axons↗
An inhibitor of cellular cytotoxicity is present in multiple sclerosis.
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.
Cytotoxicity, Immunologic↗
Familial idiopathic striopallidodentate calcifications.
We report a father and son with striopallidodentate calcifications. Metabolic studies excluded calcium/phosphorus metabolism disturbances and no specific etiology was found. The structure of the calcified areas differed, on magnetic resonance imaging, depending on location and, probably, age. There are nine families with similar clinical and radiologic backgrounds and no evident etiology in the literature. Transmission is most often autosomal dominant, and in contrast with physiologic senescent basal ganglia calcification, the prognosis appears to be poor.
Basal Ganglia Diseases↗
Hereditary motor and sensory neuropathy type II with axonal lesions.
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Adult↗
[Multiple sclerosis: current problems].
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Humans↗
Booster effect of tuberculin testing in healthy 6-year-old school children vaccinated with Bacillus Calmette-Guérin at birth in Santiago, Chile.
In order to determine whether tuberculin testing caused a booster effect in children vaccinated with Bacillus Calmette-Guérin (BCG) at birth, we studied forty 6-year-olds by repeat tuberculin testing 2 weeks later on the contralateral forearm. All children were healthy and had no known exposure to tuberculosis. None of the children had a history of mycobacteriosis other than tuberculosis. The mean induration was 2.3 +/- 1.8 mm for the first tuberculin reaction and 7.6 +/- 3.3 mm for the second tuberculin reaction (P less than 0.005). Four children had positive reactions (greater than or equal to 10 mm) to the first purified protein derivative test; 18 children were positive upon retesting. Eleven of these latter children had increases of at least 6 mm from reactions less than 10 mm to greater than or equal to 10 mm. The size of the BCG scar was significantly correlated to the size of both the first and second purified protein derivative reactions (P less than 0.01), suggesting that the increased reactivity upon retesting was a consequence of sensitization induced by BCG vaccination 6 years earlier. All children remained healthy after this study was completed. Retesting of tuberculin reactivity within 2 weeks in BCG-vaccinated children with reactions less than 10 mm will produce reactions greater than 10 mm in some healthy children who may not require antituberculosis treatment.
Antibodies, Bacterial↗
[Divry-Van Bogaert cortico-meningeal angiomatosis and Sneddon's syndrome. Nosological study. Apropos of 4 cases].
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.
Adult↗
[Neurogenic muscular hypertrophy. Association with abnormal electrophysiological activities].
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.
Adult↗