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Medical horror story
Published in Lester D. Friedman, Therese Jones, Routledge Handbook of Health and Media, 2022
A seven-year-old girl, Sadie, has mysterious seizures. She is eventually diagnosed with Rasmussen’s encephalitis, an inflammatory condition causing progressive damage to one side of her brain, marked by increasingly severe seizures and irreversible cognitive and motor damage. The indicated treatment is hemispherotomy, the surgical bisection of her brain with removal of the damaged half. This is non-fiction, presented in Netflix’s program Diagnosis. Sadie’s mother describes her response when the condition has been identified: “Once you Google Rasmussen’s encephalitis it’s pretty horrifying to read about” (“Second Opinions” 00:11:00). Her horror is different from the fear that Diagnosis purports to address by solving medical mysteries: as another patient on the show puts it, “Not knowing is the scariest thing in the world” (“Detective Work” 00:07:00). Fear is anticipatory; horror is reactive. Knowing can, Sadie’s mother suggests, be worse than fearing.
Immune Response and CNS Injury
Published in Martin Berry, Ann Logan, CNS Injuries: Cellular Responses and Pharmacological Strategies, 2019
Rasmussen’s encephalitis is a possible exception. This is a rare form of focal epilepsy associated with pronounced focal cortical inflammation. The condition may be associated with antibodies to the glutamate receptor type R375–77 and it has been suggested that it is triggered by brain damage or infection, these events opening the BBB to the circulating antibodies, although how these arise is not known (it is possible that they represent cross-reactive antibodies generated against bacterial periplasmic amino acid-binding proteins78). Interestingly, the lesion is typically unilateral and does not spread to the contralateral hemisphere even after hemispherectomy.79 Why the lesion does not spread is unclear if all it requires is a break in the BBB subsequent to an activation of microglia and perivascular macrophages. Activation of these cells following unilateral lesioning appears not to spread generally across the midline (personal observations), as is also the case with astrocyte activation.80 Similarly, following a local antigenic induction of inflammation the response spreads throughout the ipsilateral cortex but not the contralateral.28,39 It is unlikely that this asymmetric spread of activation is due to either a humoral factor alone or intrinsic neural paths, but may reflect a spread along the ipsilateral vascular tree, possibly through a paracrine mechanism via the perivascular macrophages and microglia.
Neuropathogenesis of viral infections
Published in Avindra Nath, Joseph R. Berger, Clinical Neurovirology, 2020
Avindra Nath, Joseph R. Berger
Monocytic infiltration into the brain, may have a dual purpose. The ability of activated monocytes to produce cytokines such as TNF-alpha have been associated with neurotoxic properties [38]. On the other hand, it has also been shown that monocytes can produce neurotrophic factors thus playing a neuroprotective role. Understanding the regulation of this delicate balance may be critical for therapeutic approaches that aim to target monocytic infiltration within the brain. Monocytes are unique in that they express a large number of chemokine receptors and hence respond to a lot of different chemokines. Hence, not surprisingly, they are an important participant of the cellular infiltrates in most viral infections of the brain. Some viruses such as HIV [39], measles virus [40] and human parvovirus B19 [41] can cause fusion of the monocytic cells resulting in multinucleated giant cells. Some HIV-infected patients with opportunistic CMV or VZV brain infection, may have multinucleated giant cells that are co-infected with HIV and VZV or CMV [42]. It is thus possible that the formation of multinucleated giant cells may represent a phenomenon where by one infected cell is trying to engulf another. The cellular infiltrates may organize themselves into small nodules, called microglial nodules. However, these nodules are composed not only of microglial cells and infiltrating monocytes but may have lymphocytes and reactive astrocytes as well. Microglial nodules are most often seen with HIV infection [39], some of the herpes viral infections of the brain [43–45] and with West Nile encephalitis [46]. It may also be present in Rasmussen’s encephalitis, the etiology of which remains obscure [47].
Diagnosing autoimmune encephalitis based on clinical features and autoantibody findings
Published in Expert Review of Clinical Immunology, 2019
Autoimmune encephalitides with a dominant epileptic phenotype are considered ‘autoimmune epilepsies’. This term, too, has a special history. One rare and severe form of epilepsy, Rasmussen encephalitis, was described in 1958 [15]. Several times, autoantibodies directed against different antigens have been found in Rasmussen patients. The most spectacular description was that of antibodies against the subunit 3 of the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR, GluR3 antibodies) in 1994 [16]. This and the other antibodies, however, are today regarded as bystanders of an otherwise T cell dominated encephalitis [17,18]. Nevertheless, the idea of autoantibodies potentially causing epileptic seizures by interaction with neural receptors was stimulated by this disease, and the idea of antibody-mediated ‘autoimmune epilepsy’ was proposed, even though at this time, there were not yet solid data supporting it [19]. Meanwhile, the concept of epilepsy arising from well-defined antibody mediated conditions (by not completely understood mechanisms) has been acknowledged. It has been recognized by the International League against Epilepsy in its most recent classification proposal: ‘Immune-mediated epilepsy’ is one of five defined etiological categories for the epilepsies [20]. The term ‘autoimmune epilepsy’ is increasingly used in the medical literature (Figure 1).
Motor cortex relocation after complete anatomical hemispherectomy for intractable epilepsy secondary to Rasmussen's encephalitis
Published in British Journal of Neurosurgery, 2019
Mitchell T. Foster, Kumar Das, Paul May
A previously fit and well five-and-a-half-year-old girl presented with left-sided focal seizures. These escalated in frequency and severity despite medical management. One year after presentation, left-sided hemiparesis emerged and MRI identified right hemisphere volume loss. Rasmussen’s encephalitis was diagnosed. Twenty months after presentation, aged seven, she had developed severe hemiparesis, and seizures persisted despite maximal medical therapy (Carbemazepine, Levetiracetam and Clobazam, with failed trials of Clomethiazole, Sodium Valproate and Topiramate). Speech was intact, and she was said be performing at the level of her peers academically by her home tutor, notwithstanding the seizure burden. Complete anatomical right sided hemispherectomy was therefore performed. The procedure was performed without complication, and the patient recovered well post-operatively and experienced relief of seizures.
Neurological and neuropsychiatric manifestations of porphyria
Published in International Journal of Neuroscience, 2019
Yiji Suh, Jason Gandhi, Omar Seyam, Wendy Jiang, Gunjan Joshi, Noel L. Smith, Sardar Ali Khan
Comorbidities are common among patients with porphyria. In a case study by Shen et al., a woman suffered from Rasmussen encephalitis and AIP. These diseases caused her to have refractory partial seizures and progressive hemispheric atrophy which was associated with abdominal pain, dark urine, and hyponatremia. Comorbidities like these may be difficult to treat due to the effect of drugs on porphyria. The patient required antiepileptic drugs such as phenobarbital and valproate to treat her seizures. However, problems arise because these drugs are also porphyrinogenic. Attempts to discontinue the drugs caused the seizures to worsen. In addition, brain edema and coma developed, causing the patient to not be able to take phenobarbital [44].