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Indian Journal of Psychological Medicine
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6 This manuscript has been submitted to Indian Journal of Psychological Medicine
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Journal Name: Indian Journal of Manuscript ID: SZJ-2020-0277
10 Psychological Medicine
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Manuscript Type: Letter to the Editor Manuscript Title: ADULT ONSET
12 RASMUSSEN’S SYNDROME
13 PRESENTING WITH PSYCHOSIS
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15 Keywords: Rasmussen's Encephalitis, Viral Encephalitis, Neurocysticercosis, Gliosis,
psychosis,
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Abstract: Introduction: Rasmussen’s syndrome is a rare neurological disorder of
18 inflammatory aetiology characterised by encephalitis, intractable seizures, hemiparesis,
19 variable motor deficits, and dementia that usually affects one side of the brain. It is found
20 more commonly in children than adults.
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Case History: Here we report a case of an adult man presenting with psychosis as a
sequelae of Rasmussen’s encephalitis.
22 Discussion: Rasmussen’s syndrome characteristically has three stages – a prodromal
23 stage presenting with a low frequency of seizures and mild hemiparesis; an acute stage,
24 characterised by frequent seizures with prominent neurological signs like of worsening
hemiparesis, hemianopia, and cognitive deterioration; and a residual stage with
25 irreversible cognitive and neurological damage, including aphasia.
26 Conclusion: Further studies are necessary to investigate the various psychiatric
27 symptoms manifesting in a case of Rasmussen’s syndrome and to evaluate response to
28 various psychotropic drugs.
Keywords: Rasmussen’s encephalitis, viral encephalitis, gliosis,
29 neurocysticercosis,psychosis
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Theodore Rasmussen and his colleagues first described his eponymous disease in 1958, in three
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children with a longstanding illness causing focal seizures and worsening damage to one
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cerebral hemisphere. It is a progressive disease characterised by drug-resistant focal epilepsy,
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progressive hemiplegia, and cognitive decline, with unihemispheric brain atrophy.1 It has been
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suggested that the syndrome should be divided into three clinical stages. The first is the
prodromal stage, with a median duration of seven months (range: 0 months to 8.1 years), a low
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frequency of seizures and mild hemiparesis. The acute stage, which comes next, has a median
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duration of eight months and is characterised by frequent seizures. It is accompanied by
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prominent neurological signs like of worsening hemiparesis, hemianopia, and cognitive
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deterioration. Involvement of the dominant hemisphere may lead to aphasia. The last stage is
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the residual stage with irreversible damage and less frequent seizures than in the acute stage.2,3
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Though considered as an illness in children, adult and adolescent patients account for 10 per
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cent of all cases.2 This complex case describes an adult patient who presented with psychosis
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as a sequelae of Rasmussen’s encephalitis.
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Case details
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23 A 32-year-old man came to the outpatient department accompanied by his father, with
24 complaints of withdrawn behaviour, unprovoked anger outbursts, occasional smiling to self, poor
comprehension and delayed response for last 13 years. According to the informant, 1 month
prior to the onset of symptoms, the person had high grade fever with multiple episodes
25 of sudden onset of abnormal movements of both upper and lower limbs, loss of consciousness,
26 frothing at the mouth and incontinence for which he had received inpatient treatment.
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29 The last episode of seizure was 8 years back. However, throughout these last 13 years the
30 patient had been withdrawn and did not communicate verbally with anyone, sometimes using
gestures to communicate. He was often noticed smiling or muttering incomprehensibly by
31 himself. He seemed to have poor comprehension of instructions and had a slowing of response.
32 He also had episodes of unprovoked aggression towards his family members and outsiders.
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The left upper and lower limb had hypertonia, hyperreflexia, and an equivocal plantar response.
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Ankle clonus was observed on left side. Romberg’s test was positive. There was no anisocoria,
35 abnormality of pupillary reflexes, extraocular eye movements, papilledema, or neck stiffness.
The rest of the central nervous system and systemic examination were within normal limits.
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On mental status examination, the patient was conscious but remained withdrawn throughout
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the Interview. His attention was aroused with difficulty but not sustained. He did not respond
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with words, however, he inconsistently obeyed some of the instructions given to him during
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the course of the interview. His affect was blunt; mod thought, perception, higher mental
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functions and judgment could not be assessed. The clinical differential diagnoses were those
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of intracranial space-occupying lesion like tuberculoma or neurocysticercosis, tuberculous or
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viral encephalitis.
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An MRI of the brain showed diffuse reduction of volume with thinning of cortex and
7 subcortical gliosis in right cerebral parenchyma, prominent sulci and sylvan fissure, gross
dilation of right lateral ventricle and mild shift of midline towards right side. Volume loss was
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noted in right cerebral peduncle, thalamus and left cerebellar hemisphere with thinning of folia
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and left cerebellar peduncle. (Figure 1).
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With history of fever and seizures; aforementioned clinical findings, and findings on the
12 contrast-enhanced MRI of the brain, a diagnosis of Rasmussen’s encephalitis in residual
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presenting with psychotic symptoms was made. His score was 46 on the Brief Psychiatric
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Rating Scale.
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He was started on tablet Amisulpride 100 mg twice daily. After 1 month of continuing the
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medication, the guardian of the parent reported some improvement.
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The subject of this case study most probably presented in the residual stage of Rasmussen’s
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encephalitis as he exhibited hemiparesis, aphasia and other neurological symptoms along with
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certain psychotic symptoms.
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The pathophysiology of Rasmussen’s syndrome can be divided into 4 types for convenience of
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understanding. The first type has inflammation with microglial nodules; the second type has
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complete necrosis of at least one gyral segment; third type shows neuronal loss and gliosis; and
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the fourth type exhibits gliosis and glial scarring.7,8 No laboratory test can be used to confirm
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the diagnosis of Rasmussen’s syndrome.
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An MRI of the brain in most patients with Rasmussen’s syndrome shows unilateral
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enlargement of the CSF compartment with maximum accentuation in the insular and peri-
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insular region, and increased signal intensity in the cortical, subcortical, or both regions. MRI
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findings in our patient conformed to these usual findings, however, cerebellar involvement was
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also seen, which is unusual in patients with Rasmussen’s syndrome. Other investigations like positron
30 emission tomography (PET), single photon emission computed tomography (SPECT)
and magnetic resonance spectroscopy (MRS) can be used to confirm the unilateral nature of
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suspected Rasmussen’s syndrome.2 The goals of therapy in acute stage are to decrease
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inflammation, restore functional capacity, and control seizures, and in residual stage is to
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restore some degree of functionality. The various modalities of treatment available to achieve
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these goals include pharmacological, immunotherapeutic, surgical, and rehabilitative
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techniques. 4,5,6
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The man in this case, based on clinical and MRI brain findings, fulfilled the European
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consensus diagnostic criteria for the diagnosis of Rasmussen’s syndrome.2
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Conclusion - Rasmussen’s syndrome with adult onset, presenting with psychosis is a rare
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entity. As the neurological symptoms were largely absent, management with antipsychotic for
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the psychiatric symptoms has provided some positive results. However, this needs to be further
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explored.
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Referencces
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1. Rasmussen T, Olszewski J, Lloydsmith D. Focal seizures due to chronic localized
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encephalitis. Neurology.1958;8:435–45.
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2. Bien CG, Granata T, Antozzi C, Cross JH, Dulac O, Kurthen M, et al. Pathogenesis,
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diagnosis and treatment of Rasmussen encephalitis: a European consensus statement.
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Brain.2005;128(Pt 3):454–71.
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12 3. Bien CG, Widman G, Urbach H, Sassen R, Kuczaty S, Wiestler OD, et al. The natural history
13 of Rasmussen’s encephalitis. Brain (2002) 125:1751–9.
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16 4. Terra-Bustamante VC, Machado HR, Dos Santos OR, Serafini LN, Souza-Oliveira C,
17 Escorsi-Rosset S, et al. Rasmussen encephalitis:long-term outcome after surgery. Childs Nerv.
Syst. 2009; 25:583–9.
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19 5. Bien CG, Tiemeier H, Sassen R, Kuczaty S, Urbach H, von Lehe M, et al. Rasmussen
20 encephalitis: incidence and course under randomized therapy with tacrolimus or intravenous
21 immunoglobulins. Epilepsia. 2013 Mar;54(3):543–0.
22 6.Bien CG, Szinay M, Wagner J, Clusmann H, Becker AJ, Urbach H. Characteristics and
23 surgical outcomes of patients with refractory magnetic resonance imaging-negative epilepsies.
24 Arch. Neurol. 2009; 66:1491–9.
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7. Bien CG, Elger CE, Wiendl H. Advances in pathogenic concepts and therapeutic agents in
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Rasmussen's encephalitis. Expert. Opin. Investig. Drugs. 2002; 11:981–9.
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28 8. Robitaille Y. Neuropathologic aspects of chronic encephalitis. In: Chronic encephalitis and
29 epilepsy: Rasmussen's syndrome. F. Andermann ed. Boston: Butterworth-Heinemann; 1991.
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23 Fig 1. MRI of the brain shows atrophy of right cerebral hemisphere (horizontal arrow) with dilatation
24 of the right lateral ventricle (upwards pointing arrow).
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Fig 2. MRI of the brain shows atrophy of cerebellar peduncle (upper row) as well as atrophy of
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