11/73. Acute demyelinating disease. classification and non-invasive diagnosis.Five young patients are described with biopsy-proven acute demyelinating disease. Two cases are classified as Schilder's disease, a particular childhood form of multiple sclerosis (MS) with atypical clinical manifestation, normal or atypical CSF-findings and large bilateral lesions in magnetic resonance imaging (MRI). Two further cases presented with a fulminant bout of MS with typical clinical picture and CSF-findings; they are classified as Marburg's disease. The last case was an acute second bout of classical MS. The biopsy seemed to be justified in 4 cases, but unnecessary in the last case. Localized proton magnetic resonance spectroscopy (MRS) performed in one case allowed us to examine the focal cerebral abnormalities directly and non-invasively. The spectra revealed a pattern typical of acute demyelination suggesting potential for a replacement of biopsy in the future.- - - - - - - - - - ranking = 1keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
12/73. Modern MRI tools for the characterization of acute demyelinating lesions: value of chemical shift and diffusion-weighted imaging.Acute demyelinating lesions occur in various inflammatory disorders of the CNS. Apart from multiple sclerosis, most cases can be attributed to an overshooting immunological response to infectious agents called acute disseminated encephalomyelitis (ADEM). ADEM, which is mostly characterized by a monophasic course, has a multiphasic variant (MDEM). The early application of corticosteroids has been shown to be beneficial for the outcome; thus, an early diagnosis is highly desirable. Furthermore, the differential diagnosis ruling out neoplastic disorders may be difficult using conventional MRI alone. The potential diagnostic value of advanced MR techniques such as chemical shift imaging (CSI) and diffusion-weighted imaging (DWI) was investigated in a patient with MDEM, who had a new lesion in continuity with the initial disease manifestation. CSI was performed at 1.5 T with a long echo time of 135 ms for the evaluation of N-acetyl-aspartate (NAA) and choline (Cho) and with short TE of 30 ms for macromolecules (mm) and myo-inositol (mI). DWI was performed using a single-shot isotropic EPI sequence. Whereas acute and chronic areas of demyelination were neither distinguishable on T2- nor on contrast-enhanced T1-weighted images, CSI and DWI revealed different metabolite concentrations and diffusion characteristics within the composite lesion, clearly separating acute from chronic areas of demyelination. In conclusion, the addition of CSI and DWI may add to the diagnostic power of MRI in the setting of demyelinating disorders by identifying areas of acute and chronic demyelination, even in the absence of contrast enhancement.- - - - - - - - - - ranking = 1.000764844102keywords = multiple sclerosis, sclerosis, ms (Clic here for more details about this article) |
13/73. Fatal acute encephalomyelitis after a single dose of intrathecal methotrexate.A 40-year-old Hispanic man with acute lymphoblastic leukemia was treated with a single dose of intrathecal methotrexate 12 mg for prophylaxis against leptomeningeal spread of tumor. The day after methotrexate administration, the patient complained of severe back pain and urinary retention. The diagnosis of encephalomyelitis was made on day 3 after methotrexate administration, and by day 6 mechanical ventilation was begun secondary to ascending paralysis. By day 8 the patient was comatose, with minimal signs of brain activity and little hope for recovery; on day 12 he died. Although neurotoxicity is a frequent complication of methotrexate therapy, fatal acute neurotoxicity is extremely uncommon, especially in adults. The mechanisms of methotrexate toxicity remain unclear, and no effective treatment exists to prevent its occurrence. This patient rapidly progressed from mild neurotoxicity to fatal encephalopathy after one dose of intrathecal methotrexate during his third cycle of chemotherapy. Clinicians should be aware of the signs and symptoms of neurotoxicity during treatment, as well as predisposing factors that put patients receiving methotrexate at risk for neurotoxic effects.- - - - - - - - - - ranking = 0.00076484410199505keywords = ms (Clic here for more details about this article) |
14/73. Acute disseminated encephalomyelitis associated with hepatitis a virus infection.We describe the case of a 30-month-old boy who developed acute disseminated encephalomyelitis (ADEM) after hepatitis a virus (HAV) infection and ultimately died. As far as we know, this is only the second case of HAV-associated ADEM to be reported in the literature. The child was brought to hospital with fever, lethargy and weakness of 2 days duration. He had developed jaundice, abdominal pain and malaise 2 weeks beforehand and these problems had resolved within 2 days. Neurological examination revealed lethargy, generalised weakness and positive Babinski's signs bilaterally. cerebrospinal fluid examination showed mild lymphocytic pleocytosis, increased protein and elevated anti-HAV IgM and IgG titres. serum HAV IgM and IgG titres were also elevated. Despite aggressive treatment with ceftriaxone, acyclovir and anti-oedema measures, he developed papilloedema and coma within 24 hours of admission. magnetic resonance imaging of the brain revealed diffuse cerebral oedema and multifocal hyperintensities on T2-weighted images, with most lesions in the white matter of both cerebral hemispheres. The diagnosis of ADEM was established and high-dose steroids and intravenous immunoglobulin were added to the treatment regimen. However, his clinical condition continued to deteriorate and he died on the 20th day in hospital. This case shows that HAV infection can be linked with ADEM. patients with HAV infection should be examined carefully for central nervous system symptoms during follow-up. Likewise, the possibility of HAV infection should be investigated in cases of ADEM.- - - - - - - - - - ranking = 0.00076484410199505keywords = ms (Clic here for more details about this article) |
15/73. Progressive encephalomyelitis with rigidity: a paraneoplastic presentation of oat cell carcinoma of the lung. Case report.Progressive encephalomyelitis with rigidity and myoclonus (PEWR) is a rare neurological disorder, characterised by muscular rigidity, painful spasms, myoclonus, and evidence of brain stem and spinal cord involvement. A 73-year-old white man was admitted with a 10-day history of painful muscle spasms and continuous muscle rigidity on his left lower limb. He had involuntary spasms on his legs and developed encephalopathy with cranial nerves signs and long tract spinal cord symptomatology. Brain CT scan and spinal MRI were normal. The CSF showed lymphocytic pleocytosis and no other abnormalities. EMG showed involuntary muscle activity with 2-6 seconds of duration, interval of 30-50 ms and a frequency of 2/second in the left lower limb. Anti-GAD antibodies were detected in the blood. We detected radiological signs of lung cancer during the follow-up, which proved to be an oat cell carcinoma. The patient died two weeks after the diagnosis of the cancer.- - - - - - - - - - ranking = 0.0015296882039901keywords = ms (Clic here for more details about this article) |
16/73. Post-infectious encephalomyelitis associated with mycoplasma pneumoniae and legionella pneumophila infection.A case of severe acute encephalomyelitis associated with a serological diagnosis of both mycoplasma pneumoniae and legionella pneumophila infection is reported. Serological co-positivity between these two pathogens has been reported previously, and has, in general, been attributed to cross-reactivity. This is the first documented case of co-infection using more sensitive and specific serological techniques. The potential significance of these findings is discussed in the context of the considerable problems in the interpretation of serological data.- - - - - - - - - - ranking = 0.00038242205099753keywords = ms (Clic here for more details about this article) |
17/73. Acute disseminated encephalomyelitis following plasmodium falciparum malaria caused by varicella zoster virus reactivation.Neurologic complications in the course of plasmodium falciparum infections are commonly diagnosed as cerebral malaria, but bacterial or viral meningitis may exhibit similar symptoms. One to three weeks after P. falciparum malaria, acute disseminated encephalomyelitis (ADEM) can also mimick the symptoms of cerebral malaria. We describe a 31-year-old woman with life-threatening ADEM five days after successful treatment of P. falciparum malaria. The detection of IgG and IgM antibodies in serum and cerebrospinal fluid (CSF) against multiple viruses and bacteria reflected a non-specific polyclonal B cell activation and was more confusing than helpful for diagnostic decisions. Varicella zoster virus was identified with a reverse transcriptase multiplex polymerase chain reaction in the initially obtained and frozen CSF. This case and findings from the literature indicate that P. falciparum-associated ADEM might not be immune mediated, but of infectious origin. With unclear cerebral complications during or after P. falciparum malaria, prompt initiation of empirical antiviral and antibacterial treatment in addition to antimalarials may reduce mortality.- - - - - - - - - - ranking = 0.00076484410199505keywords = ms (Clic here for more details about this article) |
18/73. Acute brucella meningomyeloencephalo-spondylosis in a teenage male.BACKGROUND: brucellosis has been known from the time of Hippocrates. In 1885 Sir David Bruce isolated the causative organism from the spleens of soldiers who had died from "malta disease" (now brucellosis). There are 4 species of brucella pathogenic to humans and each of them has a specific types of animal reservoir: B.arbotus (cattle, buffalo), B.melitensis (goats, sheep, camels), B.suis (pigs), B.canis (dogs). humans are infected when they are exposed to body fluids from an infected animal. The symptoms and signs of brucellosis are protean. Diagnosis is usually dependent on clinical features and serology or culture. OBJECTIVES: To describe a case of neurobrucellosis, raise awareness about the existence of the disease in uganda and africa in general and share our experiences in its diagnosis and management. methods: A male teenager was admitted with symptoms and signs of an acute meningo-encephalitis. He underwent clinical, laboratory and basic radiological evaluation. RESULTS: The un-incubated brucella titer was significantly reactive(1:160).Oblique-view cervical x-rays showed early osteophyte formation with encroachment on the vertebral foramina on the left hand side. A diagnosis of acute brucella meningomyeloencephalo-spondylosis was made and the patient was successfully treated using conventional therapy for brucellosis (oral doxycycline for 6weeks and IM streptomycin for 2 weeks). CONCLUSIONS: Neurobrucellosis though said to be rare, is a reality in our health units should be considered in the differential diagnosis of neurological and psychiatric illnesses. The good news is that it is curable and is responsive to drugs used for other forms of brucellosis.- - - - - - - - - - ranking = 0.0011472661529926keywords = ms (Clic here for more details about this article) |
19/73. Encephalo-myelo-radiculo-ganglionitis presenting as pandysautonomia.A 68-year-old man developed pandysautonomia with severe orthostatic dysfunction, fixed heart rate, low serum levels of norepinephrine and epinephrine, absent sympathetic skin responses, and pupillary abnormalities. CSF protein was 92 mg/dl. In spite of a good recovery following corticosteroid administration, a relapse occurred, with accompanying sensory symptoms confined to both arms. Fatal sudden cardiac arrest occurred after 4 months. autopsy revealed numerous lymphocytic infiltrates, predominantly in autonomic and sensory ganglia and, to a lesser extent, in the nerve roots, spinal cord, and brainstem without evidence for an underlying tumor. This case provides histopathologic evidence for an inflammatory etiology of panautonomic neuropathy in some patients.- - - - - - - - - - ranking = 0.00076484410199505keywords = ms (Clic here for more details about this article) |
20/73. Destructive lesions in demyelinating disease.Three cases are presented in which clinical and radiological features suggested the diagnosis of glioma but surgical biopsy revealed a demyelinating process, with tissue destruction and cyst formation in two. One patient had clinically definite multiple sclerosis. Two had probable acute disseminated encephalomyelitis. Treatment with high dose steroids is appropriate when there is clinical or investigative evidence to suggest the presence of demyelinating disease, before deciding on biopsy.- - - - - - - - - - ranking = 1keywords = multiple sclerosis, sclerosis (Clic here for more details about this article) |
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