The Challenge of Integrating Care in Dual Diagnosis; Anti-NMDA-Receptor Encephalitis; Presentation And Outcome In 3 Cases Referred For Complex Specialist Rehabilitation Services

A Carroll1,2, M Delargy1

1.The National Rehabilitation Hospital, Dublin, Ireland 2
2.University College, Dublin

Abstract

The successful implementation of an integrated care pathway (ICP) for any given condition is a challenge. Even more challenging is successful ICP implementation for individuals who have multiple co-morbidities. This is further compounded when there are dual mental health and physical disabilities that require integrated working across multiple disciplines, specialties, institutions and organisations. Anti-NMDA-Receptor encephalitis (aNMDARe) is a relatively new diagnostic entity with patients typically presenting with significant psychiatric symptoms followed by progressive neurological deterioration. In this case series, we describe 3 cases of females with aNMDARe who were referred for complex specialist rehabilitation (CSR) to The National Rehabilitation Hospital. CSR is the total active care of patients with a disabling condition, and their families, by a multi-professional team who have undergone recognised specialist training in rehabilitation, led /supported by a consultant trained and accredited in rehabilitation medicine (RM). These services provide for patients with highly complex rehabilitation needs that are beyond the scope of local services. In these cases, referral to CSR resulted in the construction of a bespoke integrated care pathway (ICP) that transcended the barriers between primary, secondary and tertiary care and across the boundaries of physical and mental health. A care pathway is a complex intervention for the mutual decision-making and organisation of care processes Rehabilitation services acted as the coordinator of services in these cases to ensure implementation of the care plan and to ensure successful transitions of care and supported local specialist and general teams in the management of these complex cases

Introduction
The successful implementation of an integrated care pathway for any given condition is a challenge. This is further compounded when there is dual mental health and physical disabilities that require integrated working across multiple disciplines, specialties, institutions and organisations. Anti-NMDA-Receptor encephalitis (aNMDARe) is a relatively new diagnostic entity with patients typically presenting with significant psychiatric symptoms followed by progressive neurological deterioration1-7. In these case reports, we describe the care journey of three patients diagnosed with aNMDARe encephalitis.

Case reports
In the first case, a 45-year-old lady with a history of depression, was admitted to psychiatric services with an acute confusional state. Her symptoms persisted and she was transferred to a regional hospital. CT brain was normal, pelvic MRI revealed an ovarian mass and CSF serology was anti-NMDAR antibody positive. She had a bilateral salpingoophorectomy and hysterectomy with histology confirming a benign teratoma. She was treated with intravenous immunoglobulin (IVIg) and steroids. She was subsequently transferred for complex specialist rehabilitation (CSR) for a programme of rehabilitation for persisting agitation and short term memory loss which were impacting on her activities (Admission Disability rating scale [DRS] 9 [moderately severe disability])8. On discharge, she was independent in activities of daily living (ADLs) with residual mild memory difficulties resulting in inability to return to work (Discharge DRS 2 [partial disability]).

In the second case, a 39-year-old female with no previous psychiatric history, presented to psychiatric services with subacute paranoia, delusional beliefs and perseverative behaviour associated with severe insomnia and episodic agitation. Her symptoms continued and she was transferred to the acute hospital. She deteriorated despite treatment and required IV fluids and nasogastric nutrition. She developed akinetic mutism with self-mutilation and periods of extreme agitation. She had seizures and developed oromandibular dyskinesia. CT & MRI brain and MRI pelvis were normal. CSF serology was anti-NMDAR antibody positive. She received IV methylprednisolone , IV immunoglobulin and IV cyclophosphamide and subsequently IV Rituximab 375mg/m2 every four weeks (8 doses total). She was transferred for CSR (Admission DRS 8 [moderately severe disability] ) . On discharge, she was independent in ADLs but had residual higher executive dysfunction with poor insight in to her difficulties (Discharge DRS 2).

The third case was a 38-year-old female with a history of depression, who presented to psychiatric services with an acute confusional state, agitation and short term memory loss. Her neuropsychiatric symptoms persisted and she was transferred to the acute hospital. CT brain was normal and CSF serology was anti-NMDAR antibody positive. MRI pelvis was normal. She was treated with Plasmapheresis and mycophenolate. She was subsequently transferred for CSRS with a significant phase of dependency for all care, with immobility and abulia (Admission DRS 17 [extremely severe disability]). On discharge, she was independent in ADLs but had ongoing issues with mood, anxiety and fatigue. She was therefore not in a position to return to work (Discharge DRS 2).

Discussion
In these cases, referral to CSR resulted in the construction of a bespoke ICP that transcended the barriers between primary, secondary and tertiary care and across the boundaries of physical and mental health. This comprised an explicit statement of the goals and key elements of evidence based care; patients’ and families expectations; the facilitation of communication among the different team members and teams and with the patients and their families; the coordination of the care process by the coordination of roles and sequencing the activities of the multidisciplinary team, the patients and their relatives; the documentation, monitoring, and evaluation of outcomes, and the identification of the appropriate resources for discharge and follow up.

Implementation of the ICP resulted in improved independence in ADLs and participation for these patients who will continue their rehabilitation in the community with health and social care support. For those with residual deficits, patients with aNMDARe require access to CSR to facilitate enhanced activity and participation. There is no clear guidance in the literature about the management of aNMDARe. Early recognition and prompt diagnosis of this illness is important. Patients can present to different specialties in different settings. Response to treatment can be delayed so there is a need for Neurological, Neuropsychiatric and Rehabilitation Medicine expertise in managing these cases until symptoms resolve. Physicians therefore need to have a basic understanding of the clinical characteristics, differential diagnosis, treatment regimens and Rehabilitation requirements of these patients.

Conflicts of Interest
The authors have no conflicts of interest to declare

Correspondence:
Áine Carroll, The National Rehabilitation Hospital, Dublin, Ireland 2.
E-mail: Aine.Carroll@NRH.ie

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