1/42. Chronic steadily progressive central and peripheral predominantly motor demyelination, involving the cranial nerves, responsive to immunoglobulins.The association of central and peripheral demyelination was reported previously. Most of the cases refer to central chronic relapsing demyelination with clinical criteria for multiple sclerosis associated with later signs of peripheral nerve involvement. Other authors, described central lesions in patients with chronic inflammatory demyelinating polyneuropathy and in guillain-barre syndrome, as a seldom occurrence. We report a patient in which a chronic steadily progressive central and peripheral predominantly motor nervous system demyelination, involving the cranial nerves, was identified. The patient improved after intravenous immunoglobulin suggesting an immune-mediated mechanism. To our knowledge this presentation was not described before.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
2/42. Predominant arm weakness in acute idiopathic polyneuritis: a distinct regional variant.Acute idiopathic polyneuritis is a heterogeneous disorder with a number of clearly established variants. We describe four patients who present with an acute neuropathy predominantly affecting the arms which we believe should be considered as a distinct variant of acute polyneuritis. In two cases a primary demyelinating process affecting both motor and sensory nerves is suggested whereas in the other two the features were of a pure motor axonal degeneration. The relationship between these neuropathies, other variants of acute idiopathic polyneuritis and multifocal motor neuropathy is considered.- - - - - - - - - - ranking = 0.044610775619694keywords = demyelinating (Clic here for more details about this article) |
3/42. optic neuritis in children.PURPOSE: To describe the clinical characteristics of optic neuritis in children, including final visual acuity and development of multiple sclerosis (MS). methods: charts were reviewed of all patients < 15 years of age who presented with optic neuritis to the Bascom Palmer eye Institute or the Miami Children's Hospital between 1986 and 1998. RESULTS: Fifteen patients were identified. There was a slight female predilection in the study group (60%), with a mean age of 9.8 years at presentation. A preceding febrile illness within 2 weeks of visual symptoms was reported in 66% of patients. Initial visual acuity ranged from 20/15 to no light perception. Involvement was bilateral in 66% of patients, and disc swelling was present in 64% of involved eyes. Of the patients who underwent magnetic resonance imaging, 33% had focal demyelinating lesions in the brain, and 63% of affected nerves were enlarged or enhanced with gadolinium. Eleven patients were treated with intravenous steroids. Final visual acuity was > or = 20/40 in 58.3% of eyes. Thirty percent of the patients had vision of finger counting or worse. Four (26%) patients developed MS. The mean age of patients with MS was 12 years, compared with 9 years in children who did not develop MS. patients with unilateral involvement had an excellent visual prognosis (100% > 20/40), but a higher rate of development of MS (75%). Two patients had positive serology for lyme disease. CONCLUSIONS: optic neuritis presents differently in children than in adults. Children typically have bilateral involvement with papillitis following an antecedent viral illness. Although visual prognosis is poorer in children than adults, the development of MS is less common in children. Children who present with unilateral involvement have a better visual prognosis; however, they also develop MS at a greater frequency than children with bilateral involvement. patients who developed MS were, on average, older at presentation with optic neuritis than those who did not develop MS.- - - - - - - - - - ranking = 0.044610775619694keywords = demyelinating (Clic here for more details about this article) |
4/42. Symptomatic paroxysmal hemidystonia due to a demyelinating subthalamic lesion.We present a case of paroxysmal hemidystonia in a patient with an isolated demyelinating lesion in the subthalamic region, involving the posterior arm of the internal capsule and extending to the subthalamic nucleus and mesencephalon, possibly due to multiple sclerosis. Compared with similar reports in the literature, in our case there was a paucity of lesions, permitting a more direct clinico-anatomical correlation. The role of the subthalamic region and basal ganglia circuitry in the genesis of symptomatic dystonia is discussed.- - - - - - - - - - ranking = 0.22305387809847keywords = demyelinating (Clic here for more details about this article) |
5/42. Hemidystonia secondary to cervical demyelinating lesions.Hemidystonia is usually associated with a structural lesion in the contralateral basal ganglia. We report a patient with definite multiple sclerosis, according to Poser's criteria, presenting with an acute-onset sustained left hemidystonia. Cranial T2-weighted magnetic resonance imaging (MRI) showed several hyperintense lesions in the centri semiovali and in the periventricular area without basal ganglia involvement. Moreover cervical spinal cord T2-weighted MRI showed two hyperintense lesions in the left posterolateral spine at C2 and C3, and one lesion in the right posterolateral spine at C4 levels. The hemidystonia improved completely after daily treatment with 1000 mg of methylprednisolone, and cervical MRI was performed after the improvement which showed that the lesions had become smaller and less intense. Finally we consider that the hemidystonia may be caused by the cervical spinal cord lesions of multiple sclerosis.- - - - - - - - - - ranking = 0.17844310247878keywords = demyelinating (Clic here for more details about this article) |
6/42. Fulminant course in a case of diffuse myelinoclastic encephalitis-- a case report.We report on a 10-year old previously healthy boy who exhibited a fulminant and nearly monophasic clinical course of demyelinating encephalitis with relapsing intracranial hypertension syndrome. Histologic examination of a diagnostic brain biopsy revealed an inflammatory demyelinating process with perivascular T lymphocytic infiltration and axonal damage reminiscent of multiple sclerosis-like lesions. In the brain, the dna of human Herpes virus 6 (HHV6) was detectable. Eleven months after the initial symptoms and on maintainance with oral steroids, MRI showed demyelination of both hemispheres as well as demyelination of the brain stem and wallerian degeneration. The boy exhibited a severe neurologic defect syndrome. The clinical and radiological course is unusual because of the asymmetric progression of the encephalitis and the extensive confluent lesions without demarcated border or enhancement of the rim after injection of gadolinium. The clinical course showed no definite steroid response. The pathogenetic relevance of HHV6 remains elusive. Although single patients with HHV6-associated encephalomyelitis have been reported, HHV6 dna is occasionally detected in brains of healthy individuals.- - - - - - - - - - ranking = 0.3836014723049keywords = inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
7/42. Progressive demyelinating neuropathy after initiation of continuous ambulatory peritoneal dialysis--report of two cases.We report on two patients who developed rapidly progressive demyelinating neuropathy within 6 to 10 weeks after the initiation of continuous ambulatory peritoneal dialysis. The neuropathy in one patient resolved after kidney transplantation while that of the other patient improved with immunosuppressive therapy. The close temporal relationship between the initiation of peritoneal dialysis and the onset of neuropathy suggests that these two events may be causally related.- - - - - - - - - - ranking = 0.22305387809847keywords = demyelinating (Clic here for more details about this article) |
8/42. Long term follow up of a hemimasticatory spasm.OBJECTIVES: To describe the clinical and neurophysiological findings in a case of hemimasticatory spasm (HMS) followed during 14 years of evolution. MATERIAL AND methods: A woman suffered from very frequent paroxysmal episodes of painful involuntary occlusion of the jaw. Neurophysiological studies were performed at the 3, 12 and 14 years after the onset of symptoms. They included a needle electromyographic (EMG) evaluation of the main jaw closing and opening muscles, the jaw reflex (JR), the masseteric silent period (MSP) and the masseteric inhibitory reflex (MIR). RESULTS: Clinical symptoms remained unchanged throughout the period of observation. Conventional EMG never disclosed neurogenic signs. Voluntary closure of the jaw systematically provoked an abnormal activity with muscle cramps characteristics, restricted to the left masseter muscle. Left JR response was normal in the first evaluation and became delayed and of reduced amplitude in the second. The MSP and MIR were abolished on the left side during the spasmodic episodes whereas they were strictly normal out of them. The MIR abnormalities showed the characteristic pattern of an efferent lesional type. CONCLUSIONS: Hemimasticatory spasm probably is the consequence of an abnormal trigeminal hyperexcitability likely induced by the demyelinating lesion of its peripheral motor pathway. The main neurophysiological abnormalities may persist unmodified over a long course of the disease and allow the differential diagnosis of HMS from oromandibular dystonia and temporomandibular dysfunction (TMD).- - - - - - - - - - ranking = 0.044610775619694keywords = demyelinating (Clic here for more details about this article) |
9/42. P-ANCA vasculitic neuropathy with 12-year latency between onset of neuropathy and systemic symptoms.BACKGROUND: The differential diagnosis of chronic progressive multifocal asymmetric neuropathies is challenging. Vasculitic neuropathies, multifocal forms of chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathies, and asymmetric lower motor neuron disorders are important considerations. CASE PRESENTATION: We report a patient with an unusually long 12-year course of nonsystemic vasculitic neuropathy prior to the development of systemic manifestations. CONCLUSION: We discuss some of the difficulties involved in the diagnosis of chronic progressive multifocal asymmetric neuropathies.- - - - - - - - - - ranking = 1keywords = chronic inflammatory demyelinating, inflammatory demyelinating, demyelinating (Clic here for more details about this article) |
10/42. Evolving spectrum of movement disorders in extrapontine and central pontine myelinolysis.Extrapontine (EPM) and central pontine myelinolysis (CPM) are rare and frequently related to rapid correction of hyponatremia. We describe a 60-year-old woman who developed an unusual evolving spectrum of movement disorders secondary to EPM and CPM following intravenous sodium replacement therapy for severe hyponatremia. She presented initially with confusion, generalized coarse postural limb tremor, myoclonic jerks and quadriparesis. Subsequently her mental state improved and her tremor and weakness resolved. Over the following months, she developed progressive painful dystonia of her facial musculature and lower limbs. This gradually became generalized and associated with choreoathethosis in her limbs. In addition, she had increasing bradykinesia and rigidity, which responded poorly to levodopa treatment. Our case illustrates that while the myelin destruction occurs during the initial insult of the osmotic demyelinating process, its delayed clinical effects resulting from ineffective reorganization of neuronal structures may be progressive, evolve with time, and difficult to treat.- - - - - - - - - - ranking = 0.044610775619694keywords = demyelinating (Clic here for more details about this article) |
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