Deciphering the puzzle: a case report of Tjalma syndrome (pseudo-pseudo Meigs' syndrome) with profoundly elevated CA-125 and pleural effusion.

Elevated CA-125 levels, polyserous effusions (such as pleural effusion, ascites, etc.) in young women with systemic lupus erythematosus (SLE) may signal pseudo-pseudo Meigs' syndrome (PPMS), after excluding other causes. We describe a 32-year-old SLE patient with recurrent bilateral pleural effusions and unexplained hypercalcemia for 10 months. Extensive evaluations revealed no infections or tumors. Cytokine analysis showed elevated interleukin (IL) levels, especially IL-6 in pleural effusion. Treatment with immunosuppressive therapy resulted in reduced cancer antigen (CA) 125 levels and decreased effusion volume, demonstrating a positive response to intervention in this case of PPMS.

Lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs' syndrome and successfully treated with hydroxychloroquine.

We herein report the first case of lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs' syndrome. Lupus-related protein-losing enteropathy and pseudo-pseudo Meigs' syndrome are extremely rare complications in patients with systemic lupus erythematosus, Both have a similar clinical course characterized by producing marked ascites, and respond to steroids in typical cases. However, in our case, steroid monotherapy was inadequate and the addition of hydroxychloroquine was effective for their treatment. Furthermore, no reports have previously confirmed elevated CA 125 levels with lupus-related protein-losing enteropathy or increased 99mTc-HSA activity with pseudo-pseudo Meigs' syndrome. In addition, we are the first to report an evaluation of the histopathology of lupus-related protein-losing enteropathy. Previously reported cases have been described as being caused by either pseudo-Meigs's syndrome or lupus-related protein-losing enteropathy as the cause of the rare pathology that causes marked pleural effusion and ascites in patients with systemic lupus erythematosus, but it has not been evaluated whether the other is co-occurring. Our case highlights that there is a potential case of overlapping lupus-related protein-losing enteropathy and pseudo-Pseudo-Meigs's syndrome. Furthermore, it is possible that patients with marked ascites with elevated CA 125 levels were mistakenly diagnosed with Meigs's syndrome or pseudo-Meigs's syndrome associated with malignant or benign ovarian tumors and underwent surgery. Clinicians should not forget SLE with pseudo-Pseudo-Meigs's syndrome as one of the differential diagnoses for marked ascites with elevated CA 125 levels.

Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil / Tjalma syndrome (pseudo-pseudo Meigs') as initial manifestation of juvenile-onset systemic lupus erythematosus

El síndrome de Tjalma o pseudo-pseudo Meigs es una entidad clínica que se presenta con derrame pleural, ascitis y elevación de CA-125 sin asociación a tumor ovárico benigno o maligno en un paciente con lupus eritematoso sistémico (LES). Tjalma describió el primer caso de un paciente con LES, ascitis, derrame pleural y elevación de CA-125. Presentamos el primer caso en una paciente pediátrica de 14 años, que se presentó con ascitis y derrame pleural refractarios a tratamiento con elevación de CA-125, sin encontrar tumor ovárico, que ameritó manejo agresivo

Tjalma syndrome or pseudo-pseudo Meigs' syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management

Tjalma syndrome (pseudo-pseudo Meigs') as initial manifestation of juvenile-onset systemic lupus erythematosus. / Síndrome de Tjalma (pseudo-pseudo Meigs) como manifestación inicial de lupus eritematoso sistémico de inicio juvenil.

Tjalma syndrome or pseudo-pseudo Meigs' syndrome is a clinical condition characterized by pleural effusion, ascites and elevated CA-125 with no associated benign or malignant ovarian tumor in a patient with systemic lupus erythematosus (SLE). Tjalma described the first case of a patient with SLE, pleural effusion, ascites and elevated CA-125. We report the first case in a 14-year old patient who presented with ascites and pleural effusion refractory to treatment and elevated CA-125, in the absence of an ovarian tumor, that warranted aggressive management.

A case of mixed connective tissue disease with pseudo-pseudo Meigs' syndrome (PPMS)-like features.

Pseudo-pseudo Meigs' syndrome (PPMS) has been reported to be a rare presentation of patients with systemic lupus erythematosus (SLE). However, such a presentation is not common in other forms of connective tissue disease. We presented a case of gross ascites, pleural effusion, and marked elevation of CA-125 level (PPMS-like features) that led to a diagnosis of MCTD. The patient responded to systemic steroid therapy.

Síndrome de Meigs y falso síndrome de Meigs: sendos casos / Meigs syndrome and false Meigs syndrome: one of each type

Presentamos un caso de síndrome de Meigs y otro de falso síndrome de Meigs. El síndrome de Meigs se diagnosticó en una mujer de 49 años, con tumor de ovario derecho (fibrotecoma), ascitis y derrame pleural izquierdo. El CA 125 fue normal. Tenía el antecedente de mastectomía radical por cáncer de mama izquierda, ductal infiltrante. Se sospechó clínicamente metástasis en ovario del cáncer de mama. El diagnóstico de síndrome de Meigs se estableció preoperatorio al conocer su ingreso en neumología por derrame pleural. La paciente tardó 1 año en acceder a la intervención. El falso síndrome de Meigs se diagnosticó en una mujer de 39 años, con tumor de ovario derecho (cáncer endometrioide sólido-cordonal), ascitis y derrame pleural derecho. El diagnóstico también fue preoperatorio. Estos síndromes deben ser conocidos por el neumólogo, con el fin de poder remitir a la paciente a ginecología para la intervención, ya que tras ella remiten la ascitis y el derrame pleural, salvo cuando se trata de un FSM con estadio avanzado de la enfermedad (AU)

A five-year analysis of botulinum toxin type A injections: some unusual features.

We analyzed patients treated during the past five years with botulinum toxin type A for strabismus and blepharospasm, reviewed our successes, failures, and unusual cases, and drew conclusions based on these treatments. Thirty-seven percent of the strabismus patients were cured, but many patients who were outside the strict definitions, still believed that they were significantly improved. A prominent feature in the treatment of strabismus was variability. Frequently, patients expected to do poorly had encouraging results. One permanent overcorrection occurred, and it converted an esotopic patient into an exotropic one with diplopia. This has persisted for 2.5 years and is the longest reported overcorrection to our knowledge. Our results indicate that larger doses of botulinum toxin produce longer spasm-free intervals in the treatment of blepharospasm. One patient receiving injections for her blepharospasm discovered that its cause was her sedative medication. This is the first reported case of a benzodiazepine inducing blepharospasm to our knowledge.

Long-term treatment of involuntary facial spasms using botulinum toxin.

Botulinum toxin, a powerful pre-synaptic neurotoxin produced by Clostridium botulinum, interferes with the release of acetylcholine from nerve terminals. Since September 1985, we have been using this toxin to treat altogether 62 patients with benign facial spasms. Most of the patients had been on drugs or psychotherapy, 2 had received alcohol injections, 2 had undergone surgery of the orbicular branch, and 2 electrocoagulation of the facial nerve. In essential blepharospasm the duration of the beneficial effect after each treatment with botulinum toxin was about 3 1/2 months. In patients with hemifacial spasm the response was clearly longer, nearly 5 months in most cases. The treatment gave the best and longest-lasting relief of symptoms in patients suffering from disturbing myokymia. Response was poorest in patients suffering from facial spasms who simultaneously had a severe psychiatric disease. The most frequent side effect was mild or moderate ptosis (22.6%). Some patients complained of dry eyes and a few cases displayed facial nerve paresis. Side effects caused by botulinum toxin injections are transient but so also, unfortunately, is the beneficial effect on facial spasms.