Cases reported "Wallerian Degeneration"

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1/13. A histopathological analysis of the human cervical spinal cord in patients with acute traumatic central cord syndrome.

    STUDY DESIGN: We have applied conventional histochemical and morphometric techniques to study the changes within the human spinal 'hand' motor neuron pool after spinal cord injury in patients who presented with acute traumatic central cord syndrome (ATCCS). OBJECTIVE: To determine whether a reduction of large alpha motor neurons at the C7, C8 and T1 spinal cord levels underlies the mechanism which causes hand dysfunction seen in patients with (ATCCS). BACKGROUND: The etiology of upper extremity weakness in ATCCS is debated and injury and/or degeneration of motor neurons within the central gray matter of the cervical enlargement has been advanced as one potential etiology of hand weakness. methods: The spinal cords of five individuals with documented clinical evidence of ATCCS and three age-matched controls were obtained. The ATCCS spinal cords were divided into acute/sub-acute (two cases) and chronic (three cases) groups depending on the time to death after their injury; the chronic group was further subdivided according to the epicenter of injury. We counted the motor neurons using light microscopy in 10 randomly selected axial sections at the C7, C8 and T1 spinal cord levels for each group. We also analyzed the lateral and ventral corticospinal tracts (CST) in all groups for evidence of wallerian degeneration and compared them to controls. RESULTS: A primary injury to the lateral CST was present in each case of ATCCS with evidence of wallerian degeneration distal to the epicenter of injury. There was minimal wallerian degeneration within the ventral corticospinal tracts. In the chronic low cervical injury group, there was a decrease in motor neurons supplying hand musculature relative to the other injury groups where the motor neurons sampled at the time of death were not reduced in number when compared to the control group. CONCLUSIONS: We hypothesize that hand dysfunction in ATCCS can be observed after spinal cord injury without any apparent loss in the number of motor neurons supplying the hand musculature as seen in our acute/sub-acute (n=2) and our chronic high injury (n=1) groups. The motor neuron loss seen in the chronic low level injury was felt to be secondary to the loss of C7, C8, and T1 neurons adjacent to the injury epicenter.
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2/13. Chronic inflammatory polyradiculoneuropathy.

    The diagnostic criteria, natural history, nerve conduction characteristics, pathology, laboratory features, and efficacy of corticosteroid treatment have been evaluated personally in 53 patients with chronic inflammatory polyradiculoneuropathy (CIP) who were followed up for an average of about 7.5 years. These were patients whose monophasic neurologic deficit had not crested by 6 months, patients with recurrences, and patients with a steady or stepwise progression. The typical features of CIP include absence of an associated disease, frequent history of preceding infection or receipt of foreign protein, and tendency to involve cranial, truncal, and proximal as well as distal limb structures and to have diffusely slow conduction velocity of peripheral nerves. The most marked slowing is often very proximal. The pathologic features include serous edema, mononuclear cell infiltrates (especially in perivascular areas, but without evidence of vasculitis), macrophage-induced segmental demyelination, and hypertrophic neuritis. If our patients are representative, complete recovery occurs only infrequently; about 60% of patients are able to be ambulatory and work, 25% become confined to a wheelchair or become bedridden, and approximately 10% die from their disease. Although the bulk of the pathologic changes affect spinal roots and proximal nerves, the brain and spinal cord may be involved also. Degeneration into linear rows of myelin ovoids is the predominant type of myelinated fiber degeneration of the sural nerve at the ankle.
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3/13. Transient detection of early wallerian degeneration on diffusion-weighted MRI after an acute cerebrovascular accident.

    We report three patients with a cerebrovascular accident studied serially by MRI, including diffusion-weighted imaging (DWI). In case 1, DWI 1 day after the onset of left frontoparietal cortical infarcts showed no abnormal signal in the left corticospinal tract. DWI 12 days after onset showed high signal in the corticospinal tract, interpreted as early wallerian degeneration. This had disappeared by 22 days after onset. In case 2, DWI obtained 7 days after the onset of a right internal capsule lacunar infarct showed high signal from the right corticospinal tract in the brainstem, which was less marked 15 days after onset. In case 3, MRI on postnatal day 7 showed a cerebral haemorrhage in the right corona radiata and high signal from the right corticospinal tract on DWI. The latter disappeared by day 23. DWI shows early wallerian degeneration; transient signal abnormalities within 2 weeks of stroke should not be mistaken for new ischaemic lesions.
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4/13. Acquired cerebral hemiatrophy: idiopathic?

    The authors report the case of a young man with acquired cerebral hemiatrophy that was progressive initially but stabilized after a few months. He did not have any seizure throughout the course of his illness. No definite cause was evident despite extensive investigations. He did have a slight increase in white cell count on a cerebrospinal fluid study done during the progressive phase of his illness suggesting that an unidentified infective or inflammatory illness may have resulted in the cerebral hemiatrophy. The authors suggest that Rassmusen's encephalitis may in fact be a spectrum with the typical descriptions representing one end. The importance of multimodality imaging in evaluating patients with cerebral hemiatrophy is discussed.
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5/13. The effects of wallerian degeneration of the optic radiations demonstrated by MRI.

    The effects of wallerian degeneration can be demonstrated by MRI as abnormal signal along the course of the degenerate fibres; they have previously been reported in the corticospinal tract. We report two cases of wallerian degeneration of the right optic radiations due to lesions of the right lateral geniculate body. The anatomy and the MRI visibility of the normal optic radiations are briefly discussed.
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6/13. pathology of the sympathetic nervous system corresponding to the decreased cardiac uptake in 123I-metaiodobenzylguanidine (MIBG) scintigraphy in a patient with parkinson disease.

    Decreased cardiac uptake in (123)I-metaiodobenzylguanidine (MIBG) scintigraphy has been adopted as one of the most reliable diagnostic tests for parkinson disease (PD) in japan. To investigate the morphological basis for this finding, we performed a detailed neuropathological study of the cardiac sympathetic nervous system of a 71-year-old autopsy-proven PD patient, who presented with a marked decrease in cardiac uptake of MIBG, just 1 year prior to death. We carefully examined the intermediolateral column at several levels of the thoracic spinal cord, the sympathetic trunk and ganglia, and the nerve plexus of the anterior wall of the left ventricle and compared the findings with those of five age-matched controls. We found that the cardiac plexus was more heavily involved than the sympathetic ganglia in this patient with PD. Our study may provide further evidence that the markedly decreased cardiac uptake of MIBG observed in PD cases represents preferential involvement of the cardiac sympathetic nerve plexus in this disorder.
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7/13. MRI findings in posttraumatic spinal cord wallerian degeneration.

    We present MRI findings in a patient who presented with a remote cervical cord injury and with onset of new symptoms. Imaging showed findings compatible with wallerian degeneration (WD) above and below the level of the original injury. We review the pathogenesis of spinal cord WD and its implications.
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8/13. Partial hypotrophy of the posterior and lateral columns of the spinal cord, representing a sequela of schistosomiasis mansoni: report of an autopsied case and a review of the literature.

    An autopsied case of schistosomiasis mansoni of the spinal cord is reported. The patient had been healthy until about five years before, when he presented with paraparesis, loss of sensibility from the pelvic girdle down and fecal and urinary incontinence which persisted until death. At autopsy there was old necrosis of most of the gray matter in the middle thoracic segment of the spinal cord and severe hypotrophy of the posterior columns in the lumbar spinal cord, of the posterior and anterior spinocerebellar tracts, and of the lateral corticospinal tract, unilaterally, in the entire thoracolumbar spinal cord from the middle thoracic segment on. In correspondence with these lesions, over one hundred schistosoma mansoni ova were found. No parasitism by S. mansoni in the brain and in the abdominal and thoracic viscera was found. This case is compared to others in the literature and a schistosomal etiology for spinal lesions is proposed. These lesions could represent a sequela of the prolonged destructive action of the granulomatous inflammatory process. The topography of the lesions, the relationship of schistosomiasis of the spinal cord to other anatomo-clinical forms of the disease, and how the spinal cord is reached by S. mansoni are commented upon.
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9/13. spinal cord degeneration associated with type II decompression sickness: case report.

    The medical history, clinical and neuropathological findings at necropsy are described in a 50-year-old male amateur diver who suffered from Type II decompression sickness, a spinal 'bend'. He survived as a paraplegic for 4 years. In the spinal cord upward wallerian degeneration in the posterior columns and downward degeneration in the corticospinal tracts was explained by multiple small and medium sized infarcts affecting the centripetal blood supply to the cord. There was preservation of a rim of subpial fibres on the surface of the posterior and lateral columns. The grey matter and nearby white matter (supplied by the centrifugal arterial supply) was unaffected.
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10/13. wallerian degeneration in a patient with Schilder disease: MR imaging demonstration.

    wallerian degeneration in the corticospinal tract was demonstrated by magnetic resonance (MR) imaging in a patient with Schilder disease. The histochemical stages of myelin breakdown that allow its demonstration by MR imaging are reviewed.
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