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PurposeCannabidiol has been licensed in the UK for those with refractory seizures associated with Lennox Gastaut Syndrome (LGS). However, characterizing LGS in adults can be challenging. Patients are frequently too agitated to tolerate EEG monitoring. Many patients are monitored by carers whose expertise may be variable; identifying seizures, particularly non convulsive manifestations such as atonic head drops or sleep-related tonic seizures, is therefore unreliable. We sought to ascertain whether those with an LGS-type syndrome – refractory multifocal epilepsy with cognitive impairment and multiple seizure types arising in childhood, would benefit from Cannabidiol. These patients did not have the typical elec- troclinical phenotype for LGS.MethodIndividual Funding Requests were made for five patients. Four had genetically defined syndromes- Alternating Hemiplegia of Childhood, Batten’s disease (CLN5), CHD2 related epileptic encephalopathy and Tuberous Sclerosis. One had Febrile Related Epilepsy Syndrome (FIRES) of unknown aetiology. Patients were commenced on adjunctive Cannabidiol between March and December 2021. All patients were prescribed Clobazam.ResultsCannabidiol at a maximum dose of 5mg/kg lead to significant seizure reduction (>50%) in 3 patients. Our patient with Tuberous Sclerosis was the only one who did not experience a reduction in seizure intensity.ConclusionCannabidiol is potentially efficacious in adult patients with severe refractory epilepsy ‘outside’ the typical LGS phenotype.

BackgroundThe longer-term trajectory of seizures associated with autoimmune encephalitis (AIE) is not clear. Recent data in NMDA AIE suggests seizures manifest early and are generally short lived, but in LGI1 AIE they occur later and continue longer. We characterised seizure semiology and associated prognosis in two cohorts of AIE patients.MethodsCase note review of 14 patients with a ‘definite’ diagnosis of anti-NMDAR AIE and 14 patients with LGI1 AIE showed the latter to occur in older adults. Patients were categorized as having seizures within one month of presentation or no seizures and the semiology was classified as focal or generalised onset.ResultsIn the NMDA group A, 3/7 patients (23%) had focal onset compared to 6/11 patients (55%) in the LGI1 group, with predominantly faciobrachial dystonic seizures. NMDA patients were more likely to be seizure-free at 6 months (9/12, 75%) compared to LGI1 patients (6/14, 43%). Similarly, much fewer NMDAR patients remained on ant-epileptic medications at 1 year compared to the LGI1 patients. Neither seizure semiology at presentation nor presence of inflammation on MRI correlated with longer term seizure trajectory in both groups.ConclusionSeizures are common presenting symptoms in these AIE groups. More NMDA patients were seizure-free at 6 months and did not require AEDs after 1 year compared to the LGI1 patients. LGI1 patients represented a more susceptible group of AIE patients to epilepsy.

Vitamin E deficiency is known to result mainly in a spinocerebellar syndrome and involvement of the peripheral nervous system occurs less commonly. Most cases of vitamin E-deficient ataxic neuropathy reported relate to a genetically-mediated cause through mutation of the alpha-tocopherol transfer protein (TTPA) gene on chromosome 8q13 [1]. Severe subacute adult-onset rapidly disabling neuropathy due to vitamin E deficiency is not to our knowledge reported. Cognitive dysfunction is uncommon in this setting. Occurrence of isolated ymptomatic vitamin E deficiency due to pancreatic exocrine insufficiency (PEI) appears exceptional.

ObjectivesTo identify predictors of poor response to cardiac based seizure detection (CBSD) VNS therapy.DesignData was collected retrospectively for patients with epilepsy who had VNS Aspire SR implanted between June 2014 and June 2017. 51 patients who reached a therapeutic level of stimulation were included. We compared those who achieved significant seizure reduction (at least 50%) with the remainder to identify potential factors predicting response.Subjects51 patients achieving therapeutic stimulation (1.5mA) with the VNS Aspire SR.MethodsWe reviewed patients’ electronic records over the period between June 2014 and June 2018.Results32 (63%) patients achieved significant seizure reduction. The following factors applied equally to both responders and non-responders: patient age, duration of epilepsy prior to VNS insertion, seizure type, the presence of a causative structural abnormality and the presence of a carer. Co-existing learning disability and/or non-epileptic attacks were commoner among poor responders. Polytherapy (3 or more AEDs) and non-compliance with medical treatment were also commoner among the poor responders; none of these factors was statistically significant.ConclusionsFactors potentially predicting response to resective surgery (including patients’ age and duration of epilepsy) do not necessarily predict response to VNS therapy. The presence of a learning disability and the extent of seizure refractoriness may influence response to VNS therapy; a larger study is needed to assess their significance.

51 years old lady admitted to local DGH with 3 months history of vomiting, diarrhoea, weight loss, pr gressive unsteadiness and worsening cognitive function. Past medical history included type 2 diabetes, hypothyroidism and cholecystectomy. She was smoking 20 cigarettes per day and didn’t drink alcohol. Her CT TAP showed fatty pancreas, MRI head and spine was normal, PET scan was negative. During hospital stay patient’s neurological symptoms deteriorated so was referred to tertiary centre. On examination was severely confused, had widespread amyotrophy and muscles weakness but no fasciculations, profound sensory loss to all modalities, marked pseudoathetosis and sensory ataxia. All reflexes were absent, plantar reflexes were flexor. Blood tests showed abnormal LFTs: GGT 654, ALP 45, ALP 191, bilirubin and amylase was normal. CSF protein was 0.7, cells were normal. NCS and EMG showed absent upper limbs sensory potentials with reduced but present sural responses in keeping with ganglionopathy. Nutritional screen showed low vitamin E 1.1 [9.5–41.5] and A 0.57 [0.99–3.35]. Faecal elastase1 was low suggestive of pancreatic insufficiency. Patient was treated with Creon and high dose vitamin E. After 8 weeks of treatment patient’s cognitive function returned to normal, power and sensation improved, body weight increased

Thunderclap headache is most commonly associated with subarachnoid haemorrhage or other acute intracranial pathology. It’s typically investigated with head imaging and lumbar puncture. We are presenting here the case of spontaneous cervical epidural haematoma manifesting as thunderclap headache. This pathology could be missed by following standard investigations of thunderclap headache and highlighting importance of through clinical history. 86 year old man presented to Emergency Department with thunderclap headache and loss of consciousness. Patient developed severe occipital headache while leaving the bath than lost consciousness. After waking up he was unable to stand up, his lower legs felt numb and weak. Headache gradually improved within 30 min. His CT head after arrival to A and E didn’t show any acute intracranial pathology. CSF was normal, xantochromia was negative. Within 48 hours patient recovered almost completely. Was able to mobilise independently and was considered fit for discharge by medical team. After neurology review MRI scan of cervical spine was organised revealing spontaneous cervical epidural haematoma.

Introduction Neuro-Behcet’s is rare, more frequent in people from the Middle East. Vasculitis of brainstem and diencephalon and venous sinus thrombosis are typical manifestations. Case report A previously well 51-year-old white British lady was admitted for a 2 day history of blurred vision, with impaired up-gaze and convergence retraction nystagmus. The symptoms resolved rapidly. MRI demonstrated hyperintensities in the diencephalon, CSF showed 10 mononuclear cells, normal otherwise. She was re-admitted eighteen months later with sudden imbalance, headache, slurred speech and right facial weakness. She reported mouth ulcers appearing in crops continuously over the past 2 years, no genital ulcers. MRI showed T2 hyperintensities in the thalami extending into the mid-brain, pons and superior and middle cerebellar peduncles. CSF showed 54 mononucleate cells, proteins 0.67 g, and raised CSF IL-6. The symptoms resolved spontaneously. Multimodal MRI one week later showed near resolution of the lesions. After excluding neoplastic, vascular and infective causes, probable neuro- Behcet’s was diagnosed according to the International Consensus Recommendations criteria (2014). The patient remains on steroids and azathioprine, and is asymptomatic at one year. Conclusions Neuro-Behcet’s can present with relapsing brainstem symptoms. The diagnosis is challenging when not all the criteria for the systemic condition are met.

GABAb receptor autoimmune encephalitis is a potentially treatable disorder characterised by seizures, memory deficits, increased anxiety and mood dysregulation. In some patients it is associated with small–cell lung cancer and with other autoantibodies. We are presenting a case of GABAb receptor autoimmune encephalitis which was associated with non–malignant lung lesion, likely inflammatory in nature. A 62 year old woman presented with recent onset depression and tonic–clonic seizures. CT head showed patchy white matter changes particularly in the left frontal lobe. Initial treatment for suspected infective encephalitis with ceftriaxone and acyclovir was withdrawn after normal results of CSF analysis and negative viral PCR. Ongoing seizures were treated with a combination of anticonvulsants. MRI brain showed an encephalitic process involving the temporal lobes (Figure 1) with corresponding change on PET scan. (Figure 2) PET also showed a small nodule in the right lung with a paratracheal lymph node (Figure 3). Diagnosis of paraneoplastic encephalitis secondary to a lung tumour was made during lung MDT. Interval scanning at two months showed an increase in size of the pretracheal lymph node. Testing for VGKC antibodies, anti–TPO, NMDAR antibodies was negative. However repeat scan at an interval of six months showed resolution of the chest changes. Patient clinically improved with treatment of the seizures but was left with significant cognitive impairment and epilepsy. Repeat MRI brain one year since symptoms onset showed resolution of the swelling of the medial temporal lobes and hippocampi with atrophy. Signal change was still present. Further antibody testing at this time revealed a raised anti GABAb antibody level. GABAb is an inhibitory receptor that has been associated with seizures and memory dysfunction when disrupted. In a recent study 15 patients with encephalitis and GABAb receptor antibodies were clinically assessed. On the basis of clinical, MRI, and EEG findings, the brain regions most affected were the hippocampi and temporal lobes. A third of patients had pathologically confirmed small–cell lung cancer. GABAb receptor autoimmune encephalitis also seems to develop without cancer association. In this respect, GABAb receptor autoimmune encephalitis is similar to other synaptic autoimmune disorders of the CNS. After excluding one non–assessable patient, nine of the ten patients who received immunotherapy and cancer treatment (when a tumour was found) showed neurological improvement, while none of the four patients who did not receive immunotherapy or whose tumour treatment was not completed showed improvement. Our patient did not receive immunomodulatory therapy as initial investigations were pointing towards paraneoplastic character of encephalitis, which is generally poorly responsive to immunomodulation. GABAb antibodies were tested several months since the onset of patient9s symptoms. Patient improved spontaneously but was left with seizures and cognitive deficit as a sequel of her encephalitis. GABAb antibody–mediated limbic encephalitis adds to the growing spectrum of autoimmune encephalitides. Seizures are a predominant symptom and initial reports suggests a strong paraneoplastic link, but as more and more patients are described the non–paraneoplastic aetiologies are likely to increase, similar to the other antibodies in this spectrum.

COVID-19 pandemic presents significant challenges in delivering safe and efficient patient care, especially during the surges. In all health care systems, provision of available critical care facilities is a scarce resource, even in normal times. Problematic is not just the limitation of physical spaces in intensive care units, but also the availability of trained personnel. The critical care model, developed in Queen Elizabeth Hospital Birmingham to cope with the surge of COVID-19 patients, is based on early implementation of an interdisciplinary approach and extensive cooperation between the branches of practice, allowing to address both challenges. The main pillars are early upskilling of non-critical care staff, creation of safe, streamlined clinical pathways, adjustment of the physical layout of critical care units and comprehensive cross-town cooperation allowing to accommodate an increased number of patients, requiring intensive care. The model was well tested in clinical practice, enabling the hospital to increase the critical care footprint by more than 200% during the pandemic’s surge between March and May 2020.