1/64. 18Fluoro-2-deoxyglucose (18FDG) PET scan of the brain in type IV 3-methylglutaconic aciduria: clinical and MRI correlations.The clinical, 18fluorodeoxyglucose positron emission tomography (18FDG PET) and the magnetic resonance imaging (MRI) brain scan characteristics of four patients diagnosed to have 3-methylglutaconic aciduria were reviewed retrospectively. The disease has a characteristic clinical pattern. The initial presentations were developmental delay, hypotonia, and severe failure to thrive. Later, progressive encephalopathy with rigidity and quadriparesis were observed, followed by severe dystonia and choreoathetosis. Finally, the patients became severely demented and bedridden. The 18FDG PET scans showed progressive disease, explaining the neurological status. It could be classified into three stages. Stage I: absent 18FDG uptake in the heads of the caudate, mild decreased thalamic and cerebellar metabolism. Stage II: absent uptake in the anterior half and posterior quarter of the putamina, mild-moderate decreased uptake in the cerebral cortex more prominently in the parieto-temporal lobes. Progressive decreased thalamic and cerebellar uptake. Stage III: absent uptake in the putamina and severe decreased cortical uptake consistent with brain atrophy and further decrease uptake in the cerebellum. The presence of both structural and functional changes in the brain, demonstrated by the combined use of MRI and 18FDG PET scan, with good clinical correlation, make the two techniques complementary in the imaging evaluation of 3-methylglutaconic aciduria.- - - - - - - - - - ranking = 1keywords = brain (Clic here for more details about this article) |
2/64. poliomyelitis-like syndrome associated with Epstein-Barr virus infection.A 20-month-old male presented with an acute clinical syndrome resembling poliomyelitis, characterized by a flaccid monoplegia, areflexia of the involved limb, and preserved sensation. Electrophysiologic studies supported a neuronopathic localization involving the anterior horn cells. Although laboratory evidence for a poliovirus infection was absent, serologic and polymerase chain reaction studies documented an active central nervous system infection with Epstein-Barr virus, indicating that a poliomyelitis-like syndrome may be produced by infectious agents other than enteroviruses.- - - - - - - - - - ranking = 2.7771385826684keywords = central nervous system, nervous system (Clic here for more details about this article) |
3/64. Virtual reality in paraplegia: a VR-enhanced orthopaedic appliance for walking and rehabilitation.spinal cord injuries (SCIs) have a profound physical, social and emotional cost to patients and their families. Obviously SCIs severely disrupt normal patterns of interaction with the environment. Firstly, the opportunities for active interaction are inevitably diminished due to motor or sensory impairment. Moreover, such problems may increase as the time since injury lengthens and the patient becomes more withdrawn and isolated in all spheres of activity. However, advances in Information technology are providing new opportunities for rehabilitation technology. These advances are helping people to overcome the physical limitations affecting their mobility or their ability to hear, see or speak. In this chapter an overview is given of the design issues of a VR-enhanced orthopaedic appliance to be used in SCI rehabilitation. The basis for this approach is that physical therapy and motivation are crucial for maintaining flexibility and muscle strength and for reorganizing the nervous system after SCIs. First some design considerations are described and an outline of aims which the tool should pursue given. Finally, the design issues are described focusing both on the development of a test-bed rehabilitation device and on the description of a preliminary study detailing the use of the device with a long-term SCI patient.- - - - - - - - - - ranking = 0.91642164238932keywords = nervous system (Clic here for more details about this article) |
4/64. Atypical dementia and spastic paraplegia in a patient with primary lateral sclerosis and numerous necortical beta amyloid plaques: new disorder or Alzheimer's disease variant?Primary lateral sclerosis (PLS) and hereditary spastic paraplegia (HSP) are clinically similar disorders in which progressive lower limb spasticity and corticospinal tract degeneration are characteristic. We report the occurrence of progressive spastic paraplegia and frontal systems dementia in a patient with postmortem features of PLS combined with moderate Alzheimer-like changes in neocortex and hippocampus. This combination of clinical and neuropathologic findings has not been described in PLS or HSP and varies from other cases in which spastic paraplegia, dementia, and Alzheimer neuropathology occurred concurrently. This 69-year-old woman developed spastic quadriplegia and dementia over 12 years. Left leg weakness progressed over 7 years to paraplegia, then quadriplegia by age 68. Sensory and cerebellar function were preserved and fasciculations were absent. dementia characterized by concrete thinking, perseveration, and impaired executive function appeared in the seventh year and remained relatively stable until 6 months before death at age 69. Degeneration of the lateral corticospinal and dorsal spinocerebellar tracts confined to the spinal cord was evident at postmortem examination. brain stem, midbrain, and cerebellum were normal. Numerous beta/A4 amyloid positive diffuse plaques (10-15/200x field) were apparent in neocortex, and neurofibrillary tangles immunopositive for paired helical filament were detected in hippocampus. This case broadens the spectrum of disorders associated with Alzheimer neuropathologic changes. The relationship between PLS, HSP, and Alzheimer's disease requires further study.- - - - - - - - - - ranking = 0.14285714285714keywords = brain (Clic here for more details about this article) |
5/64. XY sex reversal and a nonprogressive neurologic disorder: a new syndrome?We report a patient with a unique combination of clinical findings: XY sex reversal, spastic paraplegia, mental retardation, dysmorphism, and infantile-onset olivopontocerebellar hypoplasia. The phenotype of our patient did not coincide with any of the described forms of XY reversal syndromes, hereditary or sporadic spastic paraplegias, or congenital or infantile-onset cerebellar or olivopontocerebellar atrophies or hypoplasias. The disorder of this patient likely represents a genetic condition with pleiotropic effects on brain development and sex determination and adds further evidence for the heterogeneity of spastic paraplegia/infantile olivopontocerebellar hypoplasia syndromes and sex reversal syndromes.- - - - - - - - - - ranking = 0.14285714285714keywords = brain (Clic here for more details about this article) |
6/64. Complicated hereditary spastic paraplegia with peripheral neuropathy, optic atrophy and mental retardation.An 8-year old girl with a not previously described type of complicated hereditary spastic paraplegia (HSP) is presented. Spasticity in her lower limbs had already been recognized during infancy and worsened progressively. Severe delay in mental development was observed. Peripheral neuropathy and optic atrophy developed at 5 years of age. On brain magnetic resonance imaging, an abnormally thin corpus callosum was observed. Involvement of the fasciculus gracilis was suggested by somatosensory evoked potentials. To our knowledge, there has been no reported case of complicated HSP with peripheral neuropathy, optic atrophy and mental retardation so far. We postulate that our patient is a sporadic case of not previously described complicated HSP.- - - - - - - - - - ranking = 0.14285714285714keywords = brain (Clic here for more details about this article) |
7/64. An autopsy case of classical Pelizaeus-Merzbacher's disease.A girl aged 8 years and 10 months at death had shown signs of a slowly progressive neurological disease with onset in early infancy. The main clinical features were nystagmus, spastic paraplegia, amd mental deterioration. Pathological examination of the brain showed severe demyelination with perivascular preservation of mylein islands, presenting a tigroid pattern. The patient is the second case of classical Pelizaeus-Merzbacher's disease in japan proven by autopsy.- - - - - - - - - - ranking = 0.14285714285714keywords = brain (Clic here for more details about this article) |
8/64. Thoracic myelopathy complicating acute meningococcal meningitis: MRI findings.Spinal cord dysfunction is a rare complication of neisseria meningitidis (meningococcal) meningitis. We report a 17-year-old patient who had a 3-day history of fever, headache and vomiting, agitation, and unresponsiveness. cerebrospinal fluid showed a marked polymorphonuclear pleocytosis. latex particle agglutination was positive for meningococci. The patient was given intravenous antibiotics and intravenous dexamethasone. Over the next 4 days, he developed weakness of the lower extremities, with areflexia and extensor plantar responses. MRI revealed contiguous hyperintensities on T2-weighted images involving the thoracic spinal cord from T4 to T9 and 4 brain abscesses. Five months later, he recovered brain function, but the paraparesis remained. This case illustrates that myelopathy may complicate acute meningococcal meningitis, possibly due to a vasculitis, stroke, autoimmune myelopathy, or direct infection of the spinal cord. patients with myelopathy associated with acute meningitis should receive spinal MRI. In addition, meningitis should be considered in patients presenting with acute myelopathy.- - - - - - - - - - ranking = 0.28571428571429keywords = brain (Clic here for more details about this article) |
9/64. Functional recovery from paraplegia caused by syphilitic meningomyelitis.BACKGROUND: Syphilitic involvement of the nervous system can present in many different ways. We report a patient who presented with rapidly evolving paraparesis secondary to syphilitic meningomyelitis. methods: Case report. FINDINGS: cerebrospinal fluid (CSF) studies confirmed the diagnosis of neurosyphilis. Spinal magnetic resonance imaging (MRI) studies were indicative of leptomeningeal and thoracic spinal cord disease. Treatment with IV penicillin resulted in marked clinical, radiologic, and CSF improvement. MRI imaging provided documentation of spinal cord involvement and was useful in monitoring recovery. This patient's progressive neurologic improvement was monitored for 2 years and documented by periodic Functional Independence Measure scores. CONCLUSION: Recognition of this unusual complication of secondary neurosyphilis is important, because it is a treatable cause of paraparesis with potential for good recovery.- - - - - - - - - - ranking = 0.91642164238932keywords = nervous system (Clic here for more details about this article) |
10/64. Intraspinal extramedullary haematopoiesis in a patient with myelofibrosis.A 54-year-old patient with myelofibrosis developed paresis of the legs, and bladder dysfunction due to extramedullary haematopoiesis in the spinal channel. He was given palliative radiotherapy but died shortly afterwards. Although rare, the possibility of extramedullary haematopoiesis in the central nervous system should be considered when neurological symptoms appear in a patient with myelofibrosis, because good palliation is possible with timely radiotherapy.- - - - - - - - - - ranking = 2.7771385826684keywords = central nervous system, nervous system (Clic here for more details about this article) |
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