11/54. neuromyelitis optica in a child with atypical onset and severe outcome. We report on a seven-year-old boy with inflammatory relapsing-remitting CNS disease, involving the optic nerves and spinal cord, with increasingly severe sequelae after each relapse. Clinical course, neuroimaging and laboratory findings were consistent with neuromyelitis optica. biopsy of leptomeninges and underlying nervous tissue showed increased vascularization and thickened hyalinized vessel walls, reported as suggestive for neuromyelitis optica. Clinical features at onset were atypical, rendering the case highly unusual and the diagnosis tentative. ( info) |
| Several demyelinating disorders can affect children. The differential diagnosis between these diseases is usually an arduous task. Diagnostic criteria have been proposed for some of these disorders, however most of them have not yet been clinically and prospectively validated. Here we present a case of a ten year-old boy with recurrent bilateral optic neuritis and spinal cord involvement. Clinical and cerebrospinal fluid data have fulfilled diagnostic criteria for Devic's neuromyelitis optica (NMO). The differential diagnosis with multiple sclerosis (MS) has become troublesome since not only optic nerves and spinal cord were involved. In one of the relapses a left hemiparesis with facial involvement was registered. magnetic resonance imaging was also compatible with MS. This case illustrates that CNS demyelinating disorders can fulfill diagnostic criteria for more than one demyelinating disease, making the clinical judgment an important tool in the management of these patients. ( info) |
| Cases are described with Leber's optic atrophy and neurological symptoms and/or MRI lesions suggestive of multiple sclerosis. We describe a case of a young woman with Devic's neuromyelitis optica and 3460 homoplasmic mitochondrial dna mutation. ( info) |
| A case is presented with attacks of optic neuritis followed by severe myelitis 15 years later, in coincidence with a severe stressful life event. This female patient was also affected by undifferentiated connective tissue disease. This case presents some unusual and atypical findings such as: the association of a relapsing neuromyelitis optica with an undifferentiated connective tissue disease, a very long interval between optic neuritis and myelitis, and the important role of a stressful event in developing a relapse. ( info) |
| neuromyelitis optica is a clinical syndrome characterised by acute transverse myelitis plus an acute or subacute optic neuritis with or without recovery. Although once believed to be a variant of multiple sclerosis, diagnostic criteria have recently been proposed for neuromyelitis optica, making it a clinically distinct syndrome. The term gluten sensitivity refers to a state of heightened immunological responsiveness to ingested gluten in genetically susceptible individuals, as indicated by circulating antibodies to gliadin. Several neurological complications have been described associated with gluten sensitivity ranging from peripheral neuropathy and cerebellar ataxia to an increased risk of epilepsy. Although myelopathy has been described in some case reports of coeliac disease, neuromyelitis optica has never been described in association with gluten sensitivity. We describe two cases of gluten sensitivity presenting as neuromyelitis optica with no previous history of significant gastrointestinal symptoms. Gluten sensitivity was confirmed by immunological and histological studies. ( info) |
| We describe two cases of neuromyelitis optica (NMO) with clinical and radiographically confirmed features of hypothalamic involvement, in the absence of other parenchymal brain lesions. Their course is otherwise typical of Devic's form of NMO. A review of the literature identifies additional cases of NMO in which clinical features attributable to under-recognized dysfunction of the hypothalamic-pituitary axis were present. We propose that the currently accepted criteria for the diagnosis of NMO could be revisited to recognize the possibility of lesions developing within hypothalamic structures. ( info) |
17/54. Successful high dose glucocorticoid treatment for subacute neuromyelitis optica with systemic lupus erythematosus. A 54-year-old Japanese woman with a 6-year history of systemic lupus erythematosus (SLE) was admitted to our hospital suffering from acute blindness in her right eye. Her condition recovered after steroid pulse therapy, however, 18 months later she suffered from nuchal pain for 2 weeks after which right hemiparesis with urinary incontinence developed. A spinal magnetic resonance imaging (MRI) revealed cord swelling from C2 to C7. She was diagnosed with neuromyelitis optica (NMO) and intravenous steroid administrations were immediately commenced. Her condition promptly improved. This case was unique because the steroid treatment was quite effective for this case of myelitis, which had passed the acute phase. We supposed that, because most of the lesion was not necrotic or demyelinated, but rather showed edematous change caused by vasculitis based on autoimmune pathogenesis, the symptoms progressed rather gradually and improved promptly in response to glucocorticoid treatment. ( info) |
18/54. An autopsied case of neuromyelitis optica with a large cavitary cerebral lesion. We report a case of neuromyelitis optica (NMO) with a large cerebral lesion. The patient had an episode of fever and consciousness disturbance with a tumefactive frontal white matter lesion at age 43, and then repeated bilateral optic neuritis and transverse myelitis until she died at age 63. Histopathological examinations revealed that marked tissue destruction, cavities and inflammatory changes typical of NMO were seen in the cerebrum as well as the optic nerves and spinal cord. This is the first autopsied case of NMO with a tumefactive cerebral lesion that later became cavitary. ( info) |
19/54. neuromyelitis optica (Devic's syndrome) as first manifestation of systemic lupus erythematosus. Neurologic symptoms rarely occur as presenting feature of systemic lupus erythematosus (SLE). We describe a 37-year old woman who presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiologic and laboratory findings were compatible with neuromyelitis optica (NMO). Seven years after disease onset clinical and laboratory findings were diagnostic for SLE. This case illustrates that NMO may represent a first manifestation of SLE for many years. ( info) |
20/54. Devic disease with brainstem lesions. We describe a patient who suffered from an unusually severe form of neuromyelitis optica with a hyperacute time-course evolution requiring mechanical ventilation within 3 days. The patient died after 72 days and autopsy showed major spinal cord, optic nerve, and brainstem necrosis, and multifocal necrotic lesions on the cerebellum and cerebral white matter. ( info) |