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1/18. Tuberculous involvement of the oesophagus with oesophagobroncheal fistula.

    Tuberculous involvement of the oesophagus is a rare disease. Even if it is suspected, diagnosis is often difficult though dysphagia and chest pain are the most common symptoms without any other specific signs of tuberculosis. The diagnosis is based on oesophagography, oesophagoscopy, bronchoscopy, and computed tomographic scan. Suspected tuberculosis can be confirmed with histology, smear, and culture. The two most common differential diagnoses are Crohn's disease and carcinoma. The case is reported of a female patient with tuberculous involvement of the oesophagus, who developed an oesophagobroncheal fistula during steroid treatment started for suspicion of Crohn's disease. The patient was immunocompromised due to treatment with azathioprine that she was receiving for multiple sclerosis. The fistula was successfully treated by antituberculous chemotherapy alone. ( info)

2/18. Inflammatory demyelinating polyradiculoneuropathy associated with membranous glomerulonephritis and thrombocytopaenia.

    A 66-year-old man with secondary progressive multiple sclerosis presented with a flaccid areflexic tetraparesis evolving over a 6-week period. Clinical examination and subsequent investigation confirmed a diagnosis of sub-acute inflammatory demyelinating polyradiculoneuropathy (sub-acute IDP). Nephrotic-range proteinuria and thrombocytopaenia were also noted at the time of presentation-histopathological investigation of the former showed membranous glomerulonephritis as the basis for the protein loss. The IDP, glomerulonephritis and thrombocytopaenia recovered over the same time course. There have been previous reports of either glomerulonephritis or thrombocytopaenia occurring in association with IDP-there are no such reports of all three conditions occurring and resolving simultaneously. ( info)

3/18. Reversible MRI changes of hypothalamus in a multiple sclerosis patient with homeostatic disturbances.

    multiple sclerosis (MS) patients occasionally present with homeostatic disturbances suggestive of hypothalamic dysfunction; however, the hypothalamus often appears normal on imaging, apart from confirmation in a few necropsy studies. In this article we describe a Chinese woman with atypical clinical presentation. She presented with relapsing symptoms and signs consistent with hypothalamic dysfunction including hyperprolactinema, syndrome of inappropriate secretion of anti-diuretic hormone (SIADH), hypersomnolence and temperature dysregulation. Serial MRI depicted the atypical reversible changes in the hypothalamus, correlating with the observed homeostatic abnormalities. ( info)

4/18. multiple sclerosis, interferon beta and clinical thyroid dysfunction.

    The objective of this study was to investigate frequency and presentation of clinical thyroid dysfunction in patients treated with interferon beta (IFN-beta). We have collected the cases of clinical thyroid dysfunction in 700 consecutive patients receiving IFN-beta for multiple sclerosis (MS). Five patients (four women, one man) treated with IFN-beta1b developed hyperthyroidism. Three of them have secondary progressive MS, and two have relapsing-remitting MS. It was necessary to stop IFN-beta in three cases; these patients still require carbimazole after several months. In the two other cases, hyperthyroidism disappeared spontaneously. Two patients (one man and one woman) treated with IFN-beta1a developed hypothyroidism. One of them required l-thyroxine. Lastly, an increased thyroid volume without modification of thyroid hormones plasma levels was discovered in a patient receiving IFN-beta1a. Among patients treated with IFN-beta, clinical thyroid dysfunction is much rarer than laboratory thyroid dysfunction. However, this side-effect is sometimes severe. ( info)

5/18. Clinical presentation of primary progressive multiple sclerosis 10 years after the incidental finding of typical magnetic resonance imaging brain lesions: the subclinical stage of primary progressive multiple sclerosis may last 10 years.

    BACKGROUND: Subclinical multiple sclerosis (MS) has been identified incidentally at autopsy; apparently unaffected individuals with an affected twin have demonstrated magnetic resonance imaging (MRI) changes consistent with MS, and 'MRI relapses' are several times more common than clinical relapses. CASE DESCRIPTION: A 39-year-old, right-handed man underwent MRI and PET scanning in 1986 as a 'normal' control in a Parkinson's disease study, where his father was the proband. MRI indicated multiple areas of abnormal signal intensity in a periventricular and grey-white matter junction distribution. Repeated clinical evaluations over the next 10 years were unchanged until 1996, when he complained of progressive weakness of the right foot and clumsiness in the right hand. MRI now indicated a further area of high signal intensity in the right posterior cord at the level of C5/C6. There was mild pyramidal distribution weakness in the right leg with an extensor plantar response on the same side. Over the next five years there has been mild progression in weakness and fatigue and intermittent Lhermitte's phenomenon. At no stage has there been a history of relapse, cerebrospinal fluid examination was normal and evoked responses (visual and somatosensory) are normal. CONCLUSION: This case demonstrates the phenomenon of subclinical MS, unusually supported by prolonged clinical and MRI follow-up. The patient eventually became symptomatic nine years after MRI diagnosis and is following a primary progressive course. Although MRI is known to be sensitive in identifying subclinical 'attacks', the pattern illustrated here may actually be quite typical of primary progressive MS and is compatible with the later onset seen in this subgroup of patients. ( info)

6/18. Successful treatment of intrathecal morphine overdose.

    A 47-year-old woman was diagnosed with secondary progressive multiple sclerosis, and was treated with intrathecal morphine for chronic pain via a slow-release subcutaneous pump. She accidentally received a 35-ml (510 mg) bolus injection of morphine by this route, which led to status epilepticus. She was treated with continuous intravenous naloxone infusion, and with medication to control hypertension and stop the seizure activity. The outcome was excellent, and the patient returned to her neurological baseline. This report describes the complications and the successful treatment of intrathecal morphine overdose. In order to prevent these serious errors, it is vital that only care providers who are proficient with these devices perform the refilling procedure. ( info)

7/18. The therapeutic potential of botulinum toxin.

    BACKGROUND: Botulinum toxin type A (BTX-A; commercial preparation BOTOX) is most well known for its effect on muscle contraction because of the BTX binding to the presynaptic nerve terminal, inhibiting the release of acetylcholine (ACH). The therapeutic benefit of BTX-A, however, can also be isolated to pain relief alone, suggesting that BTX-A also works through additional modes of action. OBJECTIVE: This article provides insight by an experienced physician into four different case reports. Each case demonstrates the therapeutic potential of BTX-A and the possibility of a different mechanism of action for BTX other than the inhibition of ACH release. RESULTS: Four patients, each with different symptoms such as relapsing-remitting multiple sclerosis, postherpetic neuralgia, peripheral neuropathy, and severe tingling caused by herniation of cervical vertebrae at the level of C8, were treated with BOTOX, and their symptoms were alleviated. CONCLUSIONS: The BTX-A mechanism providing pain relief is hypothesized to be something other than muscle relaxation by inhibiting the release of ACH at the neuromuscular juncture, such as inhibition of the release of substance p or the blocking of autonomic pathways, etc. This article is intended to continue to keep physicians using this substance for dermatologic indications aware of the potential unsuspected effects. ( info)

8/18. disease modifying agent related skin reactions in multiple sclerosis: prevention, assessment, and management.

    BACKGROUND: The objective for this article is to highlight some of the adverse skin manifestations associated with injectable disease modifying therapy for multiple sclerosis (MS). Early identification and intervention can often lead to minimal consequences and prolonged patient tolerance and compliance with these agents. At the University of texas Southwestern Medical Center at Dallas and texas neurology in Dallas we actively follow approximately 5000 MS patients. The majority of our patients with relapsing-remitting MS (RRMS) or secondary progressive MS (SPMS) are treated with one of the currently available disease modifying agents (DMAs). Our experience with these patients, and the challenges they face in continuing long-term treatment, constitutes the basis of our proposed treatment strategies. CONCLUSION: Skin reactions in response to injectable DMA therapy in MS are generally mild. However, some reactions can evolve into potentially serious lesions culminating in infection, necrosis, and in some circumstances requiring surgical repair. ( info)

9/18. Intravenous immunoglobulin and mitoxantrone stop the progression of secondary progressive multiple sclerosis in a patient with interferon intolerance.

    A 36 year-old female patient with secondary progressive multiple sclerosis accompanied by neuropsychiatric depression and history of intolerance to beta-interferon was treated with mitoxantrone (MX) and intravenous immunoglobulin (IVIG) for 2 years. Both clinical response and magnetic resonance imaging were performed at the end. With a combination of MX and IVIG long-term treatment, her disabilities improved as measured by the Expanded Disability Status Scale (EDSS). The combination therapy was well-tolerated. It represents an alternative to treat patients who do not respond well to cytostatics alone, or in those in whom intolerance to interferons may also occur. immunomodulation with intravenous immunoglobulin stopped the progression of the disease and avoided subsequent exacerbations during 24 months. The role of high doses of immunoglobulins in the treatment of patients with secondary progressive MS deserves clinical trials to evaluate the stopping of progression of the disease in comparison to remissions induced by cytostatics, interferons and steroids. ( info)

10/18. mitoxantrone therapy in multiple sclerosis and acute leukaemia: a case report out of 644 treated patients.

    As a rare complication of mitoxantrone (MITOX) therapy in multiple sclerosis (MS), a therapy-related acute leukaemia (TRAL) may develop. The incidence is difficult to estimate, as frequently single cases are reported, up to now a total of eight MS patients. Here we report a new case out of 644 patients. This is a 45-year-old female patient with secondary progressive MS who developed TRAL after a total dose of 48 mg/m2 MITOX. The TRAL was classified as acute myeloblastic leukaemia (AML) M4eo and showed an inversion of chromosome 16 and a partial trisomy 11. Her TRAL was treated with chemotherapy followed by allogeneic bone marrow transplantation. It responded well to the transplantation, whereas the MS symptoms initially worsened but have nearly returned to the pretransplantation level. This report brings the currently published frequency of MITOX-associated TRAL in MS therapy to five in a total of 2336 treated MS patients, representing an incidence of 0.21%. ( info)
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