1/9. Eales' disease with neurological involvement. Part 2. pathology and pathogenesis. Detailed neuropathologic examination was carried out on 1 case of Eales' disease with CNS involvement, in the form of retinal vasculopathy, followed first by signs of brain stem and cerebellar disease and then by a myelopathy, with death 4 years later from retinal infection. There was mild chronic inflammation in the retina, and sub-total demyelination of one optic nerve. The brain stem and cerebellum showed extensive vasculopathy, with various stages of venous change extending from proliferation and dilatation to haemorrhage, or to thickening with hyalinisation. The perivenular brain tissue, particularly of the cerebellum, often showed demyelination, with relative axon preservation, but no inflammation. Similar, but less pronounced venopathy was seen in the dorsal cord. There was ascending degeneration of Goll's columns and descending degeneration of the lateral columns. ( info) |
| A 33-year-old man and his 32-year-old sister developed, with an interval of 2 years, an acute fatal encephalitis following an upper respiratory tract infection of unknown etiology. autopsy documented postinfectious encephalitis in both. This is the first report of postinfectious encephalitis occurring in first degree relatives, suggesting that specific genetic factors play a role in the pathogenesis of this disease. ( info) |
3/9. Disseminated hemorrhagic leukoencephalomyelitis with localized herpes simplex brain stem infection. A case of widespread hemorrhagic and perivenous demyelinative leukoencephalomyelitis complicating a localized herpes simplex virus (HSV) brain stem infection is reported in a 28-year-old man. The presence of the virus is documented immunohistochemically and ultrastructurally. The spinal trigeminal tract at the level of the medulla oblongata contained viral antigen in the neurons, glia and in the vascular walls, including a few endothelial cells. The foci of demyelination showed deposits of gamma globulins and slight inflammatory infiltrations; the virus was absent from these lesions. It is postulated that HSV entered the central nervous system through the trigeminal nerve. Focal expression of the viral antigen on the endothelium in a sensitized host was the likely precipitating factor in the hyperacute autoimmune reaction, resulting in the widespread hemorrhagic and demyelinating lesions in the central nervous system. ( info) |
4/9. Necrotic changes of the spinal cord with immune-complex-mediated disseminated vasculitis in a case of atypical allergic encephalomyelitis. A 42-year-old woman demonstrated recurrent, progressive neurological symptoms of peripheral and central nervous system damage of undefined infectious origin. Laboratory investigations showed abnormalities in the CSF and serum, suggesting subacute viral infection. Neuropathological examination revealed complete, widespread necrosis in the cervical and thoracic segments of the spinal cord with mononuclear and microglial infiltrations. There was pronounced thickening and fibrinoid necrosis of the vessel walls with mononuclear cuffs along the spinal cord. Dispersed, similar but less intensive inflammatory changes were present in the medulla oblongata, midbrain and basal ganglia. Surprisingly, there was diffuse demyelination with only slight glial and inflammatory reactions throughout the white matter of both hemispheres. The finding of coarse- and fine-grained deposits of IgG and C3 component of complement in the vessel walls of the spinal cord and vasa nervorum of cervical roots and peripheral spinal nerves, together with positive heterologous complement binding and the results of glycine-HC1 buffer elution, suggested immune-complex-mediated disseminated vasculomyelinopathy of the CNS and PNS. Consequent local ischemic changes and hypersensitivity phenomena led to frank necrosis of the cervical spinal cord and to extreme white matter demyelination in the brain. The case was diagnosed as allergic encephalomyelitis in which diffuse demyelination occurred coincidentally with spinal cord necrosis. ( info) |
| The guillain-barre syndrome (GBS) usually occurs within one month of the precipitating cause. It is the purpose of this paper to show that typical cases may, however, appear weeks to months later. We have reviewed the collected data on these cases and suggest that they provide evidence which is in favour of a humoral, rather than a cell-mediated, aetiology for GBS. ( info) |
6/9. Recurrent disseminated vasculomyelinopathy. The monosymptomatic (recurrent infantile hemiplegia) and the polysymptomatic forms of disseminated vasculomyelinopathy that follow various infections and antigenic challenge to the nervous system were seen in two cases. These cases emphasize the importance of vasculopathy as the initial and obligatory component of the postinfectious and postimmunization neurologic syndromes as well as the clinical and pathological variability of the secondary effects on the nervous system. Recurrent infantile hemiplegia occurred in the first patient. In the second patient, after two episodes of postinfectious myelinoclastic encephalopathy, concurrent acute hemorrhagic leukoencephalopathy and an acute guillain-barre syndrome following swine flu vaccination developed. ( info) |
7/9. A syndrome of arterial-occlusive retinopathy and encephalopathy. An analysis of two new cases and four previously reported cases produced evidence for a syndrome of arterial-occlusive retinopathy and encephalopathy. All six patients were women; they ranged in age from 21 to 40 years. The clinical features of this condition include multiple branch retinal arterial occlusions and encephalopathy in which behavioral and memory disturbances predominate early. hearing loss is frequent. Except for cerebrospinal fluid pleocytosis and an increased cerebrospinal fluid protein level, there are few laboratory or radiographic abnormalities. The disease may be responsive to corticosteroid therapy. There are some similarities between this syndrome and systemic lupus erythematosus but it appears to be a distinct disease entity. A comparison of the retinal findings with those described in experimental allergic encephalitis suggests that this may be a virally induced immune-mediated disease. Although only four clearly documented examples of this syndrome have been reported, we suspect that cases may have been overlooked because of failure to recognize arterial branch occlusions in the peripheral retina. ( info) |
8/9. An ultrastructural analysis of human post-infectious (allergic) encephalomyelitis. A 6-year-old boy developed post-infectious encephalomyelitis and underwent a brain biopsy (10 days after the onset of neurologic symptoms). Electron microscopic analysis of brain showed demyelinated axons, thinly myelinated axons, aberrant remyelination, and numerous phagocytes containing myelin debris. Physical stripping of myelin by pseudopodial extensions of macrophages, as reported in experimental allergic encephalomyelitis, was noted. Hypertrophic and hyperplastic astrocytes were prominent among the phagocytic cells and played an unexpectedly active role in demyelination. ( info) |
| We report a case of multiphasic disseminated encephalomyelitis (MDEM) following viral illness presenting as multiple sclerosis (MS) in a 7-year-old boy. The patients had two episodes of alternating hemiparesis and other neurologic symptoms following viral infection, which were separated by 3 years. neuroimaging studies demonstrated multiple, discrete, small nodules and large globular lesions in the cerebral white matter, basal ganglia, brainstem and cerebellar areas. Based on typical appearance of magnetic resonance imaging (MRI) and clinical manifestations including systemic symptoms such as fever, nausea, vomiting, headache and seizures followed by consciousness disturbance and other multifocal neurologic signs, the diagnosis of MDEM rather than that of MS was made. Because it is difficult to differentiate between MDEM and MS on the basis of the clinical history, the cerebrospinal fluid examination and evoked potential studies, this report emphasizes that the MRI study of the brain may provide an important clue for the diagnosis. ( info) |