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1/49. Relating axonal injury to functional recovery in MS.

    A patient was followed after the new onset of hemiparesis from relapse of MS with serial MR spectroscopy and functional MRI. The association of clinical improvement with recovery of N-acetylaspartate, a marker of neuronal integrity, and progressive reduction of abnormally large functional MRI cortical activation with movement demonstrates that dynamic reorganization of the motor cortex accompanies remission of MS. ( info)

2/49. Continuous facial myokymia in multiple sclerosis: treatment with botulinum toxin.

    Continuous facial myokymia (CFM) is an involuntary undulating, vermicular movement that spreads across facial muscles and is associated with a characteristic electromyographic pattern. It is an infrequent clinical sign that almost always occurs in intrinsic brainstem lesions, particularly in multiple sclerosis (MS). It is usually present for only a few weeks, but it may persist for long periods of time being very troublesome for patients. We report 2 cases with MS and continuous hemifacial myokymia persisting for up to 1 month which disappeared after injection of botulinum toxin. Botulinum toxin A (BTX-A) has been used successfully to treat a variety of focal dystonias and occasionally in orbicularis myokymia, but its use has not been reported in continuous hemifacial myokymia. BTX-A appears to be effective and safe for treating persistent facial myokymia in MS patients. ( info)

3/49. Acute axonal form of guillain-barre syndrome in a multiple sclerosis patient: chance association or linked disorders?

    multiple sclerosis (MS) is characterized by inflammation, demyelination and gliosis, involving the central nervous system (CNS) and commonly sparing the peripheral nervous system (PNS). Coexistence of CNS and PNS chronic demyelination has been rarely demonstrated in chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) and in MS, but the occurrence of acute polyradiculoneuropathy in a patient with MS is even more unusual. We describe the case of a woman with relapsing-remitting MS who presented with an acute severe tetraparesis. cerebrospinal fluid (CSF) examination together with neurophysiological data and sural nerve biopsy study demonstrated an axonal form of guillain-barre syndrome (GBS). It remains unresolved if the association of an axonal form of GBS and MS is fortuitous or, on the contrary, is indicative of the coexistence in some individuals of common pathogenetic mechanisms. ( info)

4/49. Oral and maxillofacial manifestations of multiple sclerosis.

    multiple sclerosis is a chronic demyelinating disease of the central nervous system which mostly affects young adults living in the northern hemisphere. It is a disease primarily found in temperate climates, being rare in the tropics and increasing in frequency with distance from the equator. canada has one of the highest prevalence rates in the world. dentists should be familiar with the clinical manifestations that affect the oral and maxillofacial areas as well as patients' general health. Three of the most frequent oro-facial symptoms include trigeminal neuralgia, trigeminal sensory neuropathy and facial palsy. dentists should also be aware of the importance of this disease in the diagnosis, treatment and prognosis of certain oro-facial lesions or conditions. This paper reviews 2 cases of multiple sclerosis, highlights its oro-facial manifestations and discusses the dental implications of the disease. ( info)

5/49. Ocular flutter associated with a localized lesion in the paramedian pontine reticular formation.

    Ocular flutter is a rare horizontal eye movement disorder characterized by rapid saccadic oscillations. It has been hypothesized that it is caused by loss of "pause" neuronal inhibition of "burst" neuron function in the paramedian pontine reticular formation (PPRF); however, there have been no imaging studies confirming such anatomical localization. We report the case of a woman with an acute attack of multiple sclerosis associated both with ocular flutter and a circumscribed pontine lesion, mainly involving the PPRF on magnetic resonance imaging. As she recovered from the attack, both the midline pontine lesion and the ocular flutter dramatically improved. This case is the first clear evidence that at least some cases of ocular flutter are due to lesions involving the PPRF. ( info)

6/49. Axonal loss in normal-appearing white matter in a patient with acute MS.

    BACKGROUND: Brain imaging studies detect abnormalities in normal-appearing white matter in patients with MS. OBJECTIVE: To investigate the histopathologic basis for these changes in autopsy tissue from a patient with MS with 9 months' disease duration and a terminal brain stem lesion. methods: The brain stem and spinal cord were analyzed ultrastructurally and immunocytochemically for axons, myelin, and activated microglia/macrophages. RESULTS: Pathologic findings were consistent with a terminal inflammatory demyelinated lesion at the cervicomedullary junction. The ventral spinal cord column, containing descending tracts, exhibited 22% axonal loss at segment C7, but grossly normal immunostaining for myelin. Confocal and electron microscopy revealed myelin sheaths without axonal content and initial stages of myelin degradation by activated microglia/macrophages among intact myelinated axons. Axonal number and appearance was normal in ascending sensory tracts. CONCLUSIONS: These studies confirm axonal degeneration in the absence of myelin loss as one histopathologic correlate to abnormal MR findings in patients with MS. ( info)

7/49. The range of multiple sclerosis associated with neurofibromatosis type 1.

    multiple sclerosis associated with neurofibromatosis type 1 (NF1) is a very rare event. Seven patients with multiple sclerosis and NF1 are described in the literature, and all were reported to have the primary progressive form of multiple sclerosis. Three new patients with NF1 that developed multiple sclerosis are described and it is shown that the range of multiple sclerosis associated with NF1 includes the relapsing forms of the disease. The risk of having both NF1 and multiple sclerosis in north east italy is higher than would be expected based on the prevalence rates of the two diseases. ( info)

8/49. optic neuritis with transient total blindness during lactation(1).

    BACKGROUND: lactation-induced blindness is a rare but devastating puerperal complication. There are few reported cases and no consistent associated disease processes historically identified. This case illustrates lactation-associated optic neuritis as an early identifier of multiple sclerosis. CASE: A nulliparous woman underwent a term vaginal delivery complicated only by chorioamnionitis. She was treated with intravenous antibiotics, which included spontaneous bacterial endocarditis prophylaxis. Her postpartum course was uncomplicated, and she was discharged on postpartum day 2 with her infant. She was readmitted on postpartum day 16 completely blind. Evaluation revealed bilateral optic neuritis. Symptoms were initiated and exacerbated during nursing. Transitory waxing and waning of her visual deficits were noted after aggressive steroid therapy and discontinuing nursing. The patient was subsequently diagnosed with multiple sclerosis of relapsing-remitting type. CONCLUSION: multiple sclerosis must be considered as an etiology for acute puerperal lactation-associated blindness when there is no clear anatomic or infectious cause. ( info)

9/49. Rapid weaning from mechanical ventilator in acute cervical cord multiple sclerosis lesion after steroids.

    IMPLICATIONS: We report a patient with multiple sclerosis (MS), who developed neuromuscular respiratory failure requiring ventilation because of a cervical cord relapse. Serial pulmonary function tests documented improvement after steroid treatment. Cervical cord or brainstem relapses should be suspected in MS patients with respiratory failure. Identification and management of this critical condition are discussed. ( info)

10/49. Balo's concentric sclerosis: surviving normal myelin in a patient with a relapsing-remitting dinical course.

    Balo's concentric sclerosis is a demyelinating disorder in which bands of demyelination alternate with concentric bands of myelin preservation. The pathogenesis of the lesion is unknown. Previous reports using modern histopathologic techniques have shown the bands of myelin preservation to be comprised of remyelinated or partially demyelinated myelin. Here we report a case of Balo's concentric sclerosis in a 24-year-old East Indian patient with a previous history of relapsing-remitting multiple sclerosis (MS). Pathologically, the bands of myelin preservation showed myelin sheaths of normal thickness, with focal areas of demyelination. The findings, taken together with those of previously reported cases, suggest that Balo's concentric sclerosis is a variant of MS, and the concentric lesion may be an intermediary form in evolution of a chronic active MS plaque. The pathogenesis of this concentric lesion may be explained by periodic suppression of demyelination in the rapidly expanding border, allowing remyelination or only transient incomplete demyelination to occur. ( info)
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