[Acute transverse myelitis in seven patients with systemic lupus erythematosus]. / Mielitis transversa aguda en siete pacientes con lupus eritematoso sistémico.

INTRODUCTION: Although the association between transverse myelitis and systemic lupus erythematosus is rather infrequent, it is important to take this form of clinical presentation into account because it is a serious complication, which can potentially be treated but even when dealt with in the early stages does not always have a good prognosis. PATIENTS AND METHODS: We conducted a retrospective review over the past 13 years of the cases that have been admitted to our specific centre for the treatment of spinal cord injuries that were diagnosed as suffering from myelitis associated with disseminated lupus erythematosus. Demographic and clinical data, together with data about acute phase and maintenance treatments, as well as the patients' progress and sequelae are described. RESULTS: The case reports of seven patients, all of whom were young females, are studied. In two cases, myelitis was the initial presentation of lupus. The main disorder was at the dorsal, followed by the cervical, levels. Only two patients had a favourable long-term progression from the neurological point of view (both managed to walk) despite acute treatment with high doses of intravenous corticoids, and regardless of the fact that cyclophosphamide was later used. CONCLUSIONS: Myelitis associated to lupus is a rare manifestation but, owing to its important functional repercussions, it must be taken into account when faced with an acute clinical picture involving the spinal cord; this is particularly the case when it occurs in young females, with or without a previous diagnosis of autoimmune disease.

Mielitis transversa aguda en siete pacientes con lupus eritematoso sistémico / Acute transverse myelitis is seven patients with systemic lupus erythematosus

Introducción. Aunque la asociación entre mielitis transversa y lupus eritematoso sistémico es bastante infrecuente, es importante tener en cuenta esta forma de presentación clínica, porque se trata de una complicación grave, con tratamiento potencial, que incluso con intervención temprana no siempre se asocia a un buen pronóstico. Pacientes y métodos. Revisamos de forma retrospectiva durante los últimos 13 años los casos que han ingresado en nuestro centro específico para el tratamientode lesión medular, con diagnóstico de un cuadro de mielitis asociada a lupus eritematoso diseminado. Se describen los datos demográficos, clínicos, el tratamiento realizado en la fase aguda y de mantenimiento, así como su evolución y secuelas.Resultados. Se estudian los casos clínicos de siete pacientes, todas ellas mujeres jóvenes. En dos de ellas la mielitis fue la forma de inicio del lupus. La afectación predominante fue a nivel dorsal y después cervical. Sólo dos pacientes tuvieron una evolución favorable a largo plazo desde el punto de vista neurológico, consiguiendo la deambulación, a pesar del tratamiento agudo con corticoides intravenosos en dosis altas, con independencia de que posteriormente se utilizase ciclofosfamida.Conclusión. La mielitis asociada al lupus es una manifestación infrecuente, pero debe tenerse en cuenta ante un cuadro medular agudo, sobre todo si éste ocurre en mujeres jóvenes, con o sin el diagnóstico previo de la enfermedad autoinmune, debido a su importante repercusión funcional

Introduction. Although the association between transverse myelitis and systemic lupus erythematosus is rather infrequent, it is important to take this form of clinical presentation into account because it is a serious complication, which can potentially be treated but even when dealt with in the early stages does not always have a good prognosis. Patients and methods. We conducted a retrospective review over the past 13 years of the cases that have been admitted to our specific centre for the treatment of spinal cord injuries that were diagnosed as suffering from myelitis associated with disseminated lupuserythematosus. Demographic and clinical data, together with data about acute phase and maintenance treatments, as well as the patients’progress and sequelae are described. Results. The case reports of seven patients, all of whom were young females, are studied. In two cases, myelitis was the initial presentation of lupus. The main disorder was at the dorsal, followed by the cervical, levels. Only two patients had a favourable long-term progression from the neurological point of view (both managedto walk) despite acute treatment with high doses of intravenous corticoids, and regardless of the fact that cyclophosphamide was later used. Conclusions. Myelitis associated to lupus is a rare manifestation but, owing to its important functional repercussions, it must be taken into account when faced with an acute clinical picture involving the spinal cord; this is particularly the case when it occurs in young females, with or without a previous diagnosis of autoimmune disease

Lesión persistente de un músculo ocular motor secundaria a la inyección de toxina botulínica / Persistent injury to an ocular motor muscle secondary to injection of botulinum toxin

No disponible

Tetraplejía espástica secundaria a intoxicación por arsénico inorgánico / Spastic quadriplegia secondary to intoxication with inorganic arsenic

No disponible

No disponible

[Sensory neuropathy and primary biliary cirrhosis]. / Neuropatía sensitiva en la cirrosis biliar primaria.

Primary biliary cirrhosis (PBC) may associate an axonal neuropathy, a somatic and autonomic neuropathy and a very infrequently sensory neuropathy (with or without xanthomata). The aim of this paper is to describe the case of a 46 year old man diagnosed with PBC in stage I-II and a progressive sensory neuropathy (axonopathy) confined to his upper limbs with distal predominance. It had progressed slowly an began asymmetrically. A complete clinical study excluded other causes of neuropathy. We followed him clinically and electromyographically and he remains stable after two years evolution. The sensory neuropathy in this case, a primary biliary cirrhosis, is compatible with an assymetric sensory neuropathy limited to the upper limbs with assymmetric beginning.

Neuropatía sensitiva en la cirrosis biliar primaria / Sensory neuropathy and primary biliary cirrhosis

La cirrosis biliar primaria (CBP) puede cursar con neuropatía axonal, neuropatía somática y autonómica y muy infrecuentemente con una neuropatía sensitiva (xantomatosa o no).El objetivo de este trabajo es presentar el caso de un varón de 46 años diagnosticado de CBP en estadio I-II y con una clínica de 2 años de evolución de neuropatía sensitiva progresiva axonal, de predominio distal, limitada a las extremidades superiores y de características asimétricas. Mediante un completo estudio se descartaron otras causas de neuropatía. El paciente permaneció estable tras 2 años de evolución desde el punto de vista neurológico y de las alteraciones neurofisiológicas. Concluimos que la neuropatía sensitiva en este caso de CBP es compatible con una neuronopatía sensitiva de comienzo asimétrico y limitada a las extremidades superiores (AU)

[Acute transverse myelitis in systemic lupus erythematosus]. / Mielitis transversa aguda en el lupus eritematoso sistémico.

Acute transverse myelitis as complication of systemic lupus erythematosus is a known and well-characterized although uncommon clinical entity. We report here four cases of lupic myelitis collected at our hospital in the last few years and review the available literature of the last ten years, approximately the time when NMR became generally available. The clinical picture can be very variable and therefore, when facing a picture of acute myelitis, lupus should be included in the differential diagnosis; biochemistry evaluating the lupus "activity" is of poor diagnostic value, nuclear magnetic resonance is not conclusive for the etiologic diagnosis of myelitis and its prognosis has improved with therapy including pulses of steroid and immunosuppressant agents.

[Devic's optic neuromyelitis: analysis of 7 cases]. / Neuromielitis óptica de Devic: análisis de siete casos.

INTRODUCTION: Devic's optic neuromyelitis is an unusual condition characterized by the association of unilateral or bilateral optic neuritis and myelitis, both of which are relapsing. Prognosis is usually poor, both for the optic nerve and for the spinal medulla which becomes cavitated. This leads to severe handicap and deterioration in quality of life, except in cases presenting as children. PATIENTS AND METHODS: We studied seven cases of this condition and describe their clinical and neuroimaging features, cerebrospinal fluid (CSF), evaluation, complications, prognosis and treatment. This data was compared with that in the literature. RESULTS AND CONCLUSIONS: 1. It is an unusual condition--only 7 cases have been seen at our centre--making up approximately 0.1% of the inflammatory pathology of the spinal cord seen. 2. During the first few hours or days the clinical and radiological findings may not correspond. There may be marked deterioration of CNS function but with normal neuroimaging findings. This may lead to serious problems in diagnosis, which can usually be resolved by repeating the investigation. 3. Differential diagnosis should be made with intramedullary tumors when on neuroimaging there is increased spinal cord diameter with uptake of contrast, and psychiatric disorders when this is normal. 4. The condition usually deteriorates leading to severe disability, since loss of visual acuity is added to the severe spinal cord lesion. 5. Diagnosis should be made by application of the criteria of clinical evolution, imaging and biochemistry defined in the literature, although firm diagnosis can only be made on anatomopathological studies, usually at autopsy.

Treatment of chronic ventilatory failure using a diaphragmatic pacemaker.

We present our series of patients with chronic ventilatory failure treated with electrophrenic respiration: 13 males and nine females with a mean age of 12 +/- 11.5 years. The etiology was, 13 tetraplegia, five sequelae of surgical treatment of intracranial lesions, and four central alveolar hypoventilation. The mean duration of the conditioning period were 3-4 months. Eighteen patients (81.8%) achieved permanent, diaphragmatically-paced breathing with bilateral stimulation and in four (18.2%) patients, pacing was only during sleep. Five patients died (22.7%): two during the hospital stay and three at home; two deaths had unknown cause and three were due respectively to, lack of at-home care, recurrence of an epidermoid tumor, and sequelae of accidental disconnection of the mechanical ventilation before beginning the conditioning period. Two cases were considered failures: One patient had transitory neurapraxia lasting 80 days, and the other had an ischemic spinal cord syndrome with progressive deterioration of the left-side response to stimulation. One patient had right phrenic nerve entrapment by scar tissue and four suffered infections. The follow-up periods since pacemaker implantation are currently: 1, 11 years; 4, 10 years, and 17, less than 5 years. The results of our experience demonstrate that complete stable ventilation can be achieved using diaphragmatic pacing and that it improves the prognosis and life quality of patients with severe chronic respiratory failure.

[Spinal cord sarcoidosis: diagnostic and therapeutic problems]. / Sarcoidosis de la médula espinal: problemas diagnósticos y terapéuticos.

Three cases of sarcoidosis with spinal cord involvement are reported. The diagnosis in each case was very difficult, being mistaken with tuberculosis in two of the patients and with paraneoplasic myelopathy in the other one. Initial treatment was incorrect for this reason. MRI without contrast was normal, but showed the existence of lesions in two cases after gadolinium administration. Symptoms improved in two patients with prednisone, but they got worse after tapering dose. Spinal sarcoidosis is very rare, but its diagnosis most be considered and MRI with gadolinium should be performed in subacute or chronic myelopathies. Treatment with prednisone should be prolonged.

[Acute nontraumatic myelopathies: a review of 36 cases]. / Mielopatías agudas no traumáticas: una revisión de 36 casos.

The objective of this investigation was to know the features of acute nontraumatic myelopathies in our area, given the paucity of reported data. Therefore, 36 episodes of myelitis observed during a 15-year period at Hospital Nacional de Parapléjicos were retrospectively reviewed. Cases associated with multiple sclerosis were excluded. On etiological basis these disorders were classified in vascular (in its turn subclassified in surgical and non-surgical) and inflammatory myelopathies. The functional prognosis was poor. Two patients with non-surgical vascular myelitis suffered a second episode of spinal ischemia which deteriorated their conditions. The usefulness of NMR seems to be limited.

[Acute transverse myelitis and primary antiphospholipid syndrome]. / Mielitis transversa aguda y síndrome antifosfolípido primario.

The case of a 39 years old woman with acute transverse myelitis manifested as a syndrome of the anterior spinal artery is presented. Etiologic investigation diagnosed a primary antiphospholipid syndrome because of the finding of significantly high titers of anticardiolipin antibodies discarding the presence of systemic lupus erythematosus for the lack of sufficient diagnostic criteria. The association between both clinical pictures is infrequent.