Dural arteriovenous fistula presenting with recurrent focal status epilepticus and lateralised periodic epileptiform discharges.

BACKGROUND: Dural arteriovenous fistulae (dAVF) are relatively infrequently encountered, and status epilepticus (SE) and lateralised periodic discharges (LPDs) on electroencephalography (EEG) have only rarely been associated with these arteriovenous malformations. METHODS: We present a patient with recurrent presentations with focal SE, aphasia and other focal deficits of cortical function and ictal and peri-ictal LPDs on serial EEG, who was shown to have a left hemispheric dAVF associated with left transverse and sigmoid sinus thrombosis. Seizures proved refractory to four anti-seizure medications but became more amenable to control after successful embolisation of the dAVF, with subsequent resolution of the focal cortical deficits. We discuss the co-occurrence of SE and LPDs with dAVF and review previously reported cases with this rare association. CONCLUSIONS: Our report supports a causative relationship between dAVF and focal SE, manifesting as ictal LPDs on EEG, and highlights the importance of active dAVF management in achieving seizure control. The relatively good patient outcome contrasts to the few similar case reports. Whilst rare, it is important to consider dAVF as a potentially treatable condition underlying new-onset seizures, including SE.

Vaccine-Induced Functional Neurological Disorders in the Covid-19 Era.

The large amount of information available to the public regarding vaccines against Covid-19 coupled with pandemic stress and increased somatic attention could potentially precipitate development of functional neurological disorders (FNDs) following vaccination. A growing number of reports indicate that functional symptoms may follow Covid-19 vaccination, similar to those observed with other vaccines previously. We review previously reported cases of FND following vaccination against Covid-19 and present three additional cases. While two patients presented to the Emergency Department with functional movement disorders, one patient presented with protracted limb weakness and sensory dysfunction. The superficial resemblance to Guillain-Barré syndrome, a known but uncommon complication of vaccination prompted an extensive workup. Clinicians need to convey the diagnosis of FND in clear and unequivocal terms to facilitate institution of appropriate therapy and rehabilitation, but importantly also to dispel any doubts in the minds of the public regarding the safety of the available vaccines. Given the presence of significant vaccine hesitancy in many countries, this is critical to the success of the global immunisation effort.

Multifocal neuropathy and Horner's syndrome due to disseminated pyomyositis in an intravenous drug user.

BACKGROUND: The formation of abscesses with necrosis within large, striated muscles leads to pyomyositis, a condition relatively rarely encountered outside the tropics. Intravenous drug users and other immunocompromised individuals are predisposed toward this infection, which may occur due to local or haematogenous spread of infection to skeletal muscles previously damaged by trauma, exercise, or rhabdomyolysis. METHODS: We report a young male intravenous drug user with rhabdomyolysis due to use of a synthetic opioid, in whom disseminated pyomyositis was detected following evaluation for sciatic and radial neuropathies and Horner's syndrome and review available reports of peripheral nerve dysfunction in the setting of this uncommon infection. We searched online databases to identify all published reports on adult patients with pyomyositis complicated by peripheral nerve dysfunction. CONCLUSIONS: Peripheral nerve dysfunction may rarely occur via local spread of infection or compression from abscesses.

Amyotrophic lateral sclerosis with demyelinating neurophysiology and a motor band sign.

We describe an unusual case of clinical amyotrophic lateral sclerosis (ALS) with initial neurophysiological studies suggesting demyelination, along with neuroimaging findings that helped to support the eventual diagnosis. An otherwise well 68-year-old man had 8 weeks of left upper limb weakness. On examination, there were widespread lower and upper motor neurone findings suggesting ALS. However, nerve conduction studies identified sensorimotor demyelinating changes suggesting chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a diagnosis further supported by cerebrospinal fluid analysis. MR scan of the brain revealed a 'motor band', a feature seen commonly in ALS. His condition was refractory to immunotherapy with clinical progression in-keeping with ALS, establishing the diagnosis. ALS is rarely associated with demyelinating neurophysiological changes resembling CIDP. The clinical phenotype is crucial to support the correct diagnosis and imaging findings may help.

Hemidystonia-hemiatrophy syndrome following placement of internal carotid artery flow diverter stent.

We report hemidystonia and cerebral hemiatrophy in a 39-year old woman beginning three months after placement of an internal carotid artery flow diverting stent for supraclinoid aneurysm, complicated by early post-procedural stroke. We highlight the unusual demographics and short latency to onset, as well as the occurrence of striatal mineralisation, which may reflect ongoing neurodegeneration, but has also been suggested to contribute to oxidative neuronal injury. The late age of the inciting cerebral insult and of onset of the movement disorder, temporal course, and relation to endovascular intervention serve to expand the spectrum of this unusual condition.

De Novo Onset of Myasthenia Gravis after Kidney Transplantation.

Myasthenia gravis occurring de novo after kidney transplantation is a rare course of severe muscle weakness. A 57-year-old female on treatment with peritoneal dialysis following polycystic kidney disease received a renal transplant with standard basiliximab induction. She had no preceding history of neuromuscular problems. Three months after transplant she presented with progressive weakness and fatigability, finally needing a wheelchair to mobilise. Graft function was stable. Examination revealed patchy limb weakness, worsening on repeated exercise. There were no abnormalities in cranial nerves, reflexes, or sensation. Electromyography was normal, but repetitive nerve stimulation studies showed a postsynaptic neuromuscular transmission defect suggestive of myasthenia gravis. Serological testing revealed no putative antibodies. Initial treatment with pyridostigmine was not tolerated. Following an episode of hospitalisation with severe limb weakness, she received intravenous immunoglobulin and showed dramatic improvement, which persisted over the next few weeks. Approximately 6 months later, she had a relapse of her symptoms, which once again responded to intravenous immunoglobulin therapy. De novo myasthenia gravis after transplantation is a rare entity, infrequently reported in the literature. This illness is surprising since immunosuppression after transplant is usually sufficient to prevent immune-mediated disease. This patient had no history of similar illnesses. Delayed physical recovery after major surgery such as renal transplantation is often attributed to other causes such as deconditioning, and patients are often prescribed physiotherapy as a response. In this patient, the profound unexplained weakness that persisted for several weeks after transplant prompted referral to the neurologist, which enabled this rare diagnosis to be made. This story highlights the need to monitor unexpected symptoms closely and to consider a wide differential diagnosis when improvement after transplant is not along usual expected lines. Finally, this case also illustrates the benefits of multidisciplinary involvement in the care of these complex patients.

Inflammatory Cerebral Amyloid Angiopathy: A Broad Clinical Spectrum.

Cerebral amyloid angiopathy (CAA) is a common central nervous system (CNS) vasculopathy, which in some cases is associated with subacute encephalopathy, seizures, headaches, or strokes due to vascular inflammation directed against vascular amyloid accumulation. The pathological subtypes of inflammatory CAA include CAA-related inflammation (CAAri) with mostly perivascular lymphocytic infiltrates, or amyloid-beta (Aß)-related angiitis (ABRA) with transmural granulomatous inflammation. CAAri and ABRA probably represent part of the spectrum of CNS vasculopathies, intermediate between CAA and primary CNS vasculitis, and they are closely related to Aß-related imaging abnormalities and other manifestations of an inflammatory response directed against Aß in the leptomeninges and cerebral parenchyma. As treatment strategies in Alzheimer's disease shift toward potentially effective antiamyloid immunotherapy, the incidence rate of inflammatory CAA (which is probably an underrecognized condition) is likely to increase. Its clinical features are varied and include subacute encephalopathy, behavioral symptoms, headaches, seizures, and focal neurological deficits, which necessitate a high degree of suspicion for this disorder that often responds to treatment. The recent definition of the typical clinical and radiological syndrome has increased its recognition and may eliminate the need for invasive histological sampling in at least some affected patients. Here we review the pathophysiology, clinical spectrum, and approach to diagnosis, and discuss illustrative cases that highlight the wide range of clinical presentations.

Sporadic Creutzfeldt-Jakob disease in Northern Tasmania.

Creutzfeldt-Jakob disease is a rare and incurable form of rapidly progressive neurodegenerative disease. The disease is fatal, and most patients die within 1 year of diagnosis. Clinical features include progressive cognitive dysfunction, delusions or hallucinations, cerebellar ataxia, myoclonus, visual disturbances, extrapyramidal signs and eventually akinetic mutism. Most patients present with varied clinical presentation, hence making it difficult to diagnose at an early stage. We report five cases of sporadic Creutzfeldt-Jakob disease presenting to a Tasmanian hospital in Australia over a period of 52 months. We highlight significant clinical features in all our patients including few atypical presentations, emphasise on relevant clinical biomarkers and illustrate characteristic abnormalities on electroencephalogram and neuroimaging.

Inflammatory demyelinating polyneuropathy after the ChAdOx1 nCoV-19 vaccine may follow a chronic course.

BACKGROUND: Rare autoimmune neurological events have been reported during the ongoing global drive for mass vaccination as a means of controlling the Covid-19 pandemic. Guillain-Barré syndrome, an acute inflammatory neuropathy well recognised as a rare complication of influenza vaccination, has been reported to follow administration of the ChAdOx1 nCoV-19 (AstraZeneca) vaccine. METHODS: We report four patients with inflammatory demyelinating polyneuropathy after vaccination in whom a relapsing or progressive course indicated the development of chronic inflammatory demyelinating polyneuropathy (CIDP). CONCLUSIONS: Awareness of this complication and distinction from Guillain-Barré syndrome enables the timely institution of maintenance immunomodulatory treatment. Our report also highlights the likely relationship between vaccination and the subsequent development of CIDP, but definitive demonstration of a causal link needs larger studies.

Perilesional gliosis around solitary cerebral parenchymal cysticerci and long-term seizure outcome: a prospective study using serial magnetization transfer imaging.

PURPOSE: Epilepsy following solitary cerebral cysticercosis (SCC) is possibly caused by perilesional gliosis, best visualized on magnetization transfer imaging (MTI).This study aims to describe development of gliosis around SCC by prospective serial MTI and to correlate this gliosis with long-term seizure outcome. METHODS: We randomized 123 patients with SCC and new-onset seizures to treatment with albendazole plus antiepileptics (treatment), or antiepileptics only (control), and performed magnetic resonance imaging (MRI) scans at 0, 3, 6, 12, and 24 months. Prospective follow-up data regarding seizure outcome up to 5 years later were collected. MRI studies were analyzed for lesion characteristics and perilesional magnetization transfer (MT) hyperintensity. KEY FINDINGS: Clinical and radiologic data of 77 patients were analyzed. Demographic and seizure characteristics were similar in treatment and control groups. Clinical data were available up to 64 months after enrollment. At 12 months, 89.5% patients were seizure-free. MTI is more sensitive than routine imaging for detection of perilesional gliosis. Albendazole treatment did not affect imaging or clinical outcome, including development of gliosis. Independent of duration of follow-up, gliosis was associated with more seizures, and with seizure recurrence at 12 months; duration of seizures and antiepileptic therapy was longer. Gliosis was not dependent on seizure type or stage of degeneration at enrollment or persistence/calcification of the lesion. SIGNIFICANCE: Perilesional gliosis around SCC helps prognosticate seizure outcome. Poorer outcome in patients with persistent lesions is likely to be related to mechanisms other than gliosis. The lack of effect of albendazole on seizure outcome may be due to its inability to decrease formation of gliosis.

AstraZeneca COVID-19 vaccine and Guillain- Barré Syndrome in Tasmania: A causal link?

The emergence of the coronavirus 2019 (COVID-19) pandemic has presented an unprecedented global challenge. Vaccines against COVID have been developed to date. Covid-19 has been linked with the development of Guillain-Barre Syndrome (GBS), a rare immune-mediated demyelinating neuropathy. We report three cases of Guillain-Barre Syndrome and one case of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), presenting to a Tasmanian hospital, and review 15 other reported cases and discuss likely immunopathology. Nearly all reported cases of post-COVID-19 vacciation inflammatory demyelinating polyneuropathy are linked to AstraZeneca vaccination and a variant with bifacial weakness is the most reported form of GBS globally.

New-onset tic disorder following circumscribed brain injury.

Adult-onset tics represent either a secondary tic disorder ("tourettism") or a late presentation of childhood tics, which may have been previously unrecognised. Head trauma has been recognised as an infrequent cause of adult-onset tic disorder, which exhibits variable temporal relationship to the inciting injury and response to therapy. We present a patient who presented with late-onset tics seven years after a circumscribed brain injury, responding well to antidopaminergic treatment. A review of all the previously reported cases of post-traumatic tic disorder is provided. Our patient is unusual in that the injury presumed to be responsible for the development of tics was of a very focal nature, akin to previously described tic disorder following vascular insults. We discuss the rare occurrence of tourettism after such focal brain lesions and analyse the insights this provides into the anatomical substrates underlying tic disorders.

Randomized controlled trial of albendazole in new onset epilepsy and MRI confirmed solitary cerebral cysticercal lesion: effect on long-term seizure outcome.

No trials to date have focused on long-term seizure outcome in solitary cerebral cysticercal lesion (SCCL), which is believed to produce a relatively benign form of epilepsy. This is a prospective randomized controlled study to evaluate the effect of Albendazole on long-term seizure outcome in patients with MRI-confirmed solitary cerebral cysticercal lesion (SCCL). One hundred and twenty-three patients with new-onset seizures and SCCL on contrast MRI were randomized to treatment with albendazole and followed for up to five years with serial MRI and clinical evaluation. At final analysis 103 patients (M-54, F-49) with a mean age of 18.6+/-10.7 years and follow-up period more than 12 months were included. The mean follow-up duration was 31.4+/-14.8 months (12-64). At one month follow-up more patients receiving albendazole were seizure-free (62% versus 49% for controls). Subsequently there was no significant difference in overall seizure outcome between the two groups. There was no correlation between seizure semiology, albendazole therapy and long-term seizure outcome. Baseline MRI showed active lesions in all; 23% remained active at 12 months with no difference between the albendazole and control groups. Patients whose lesions resolved at 12 months showed better seizure outcome. Reduction in mean cyst area was greater in the albendazole group as compared to the controls and the difference at six months was significant (p<0.05). At three months follow-up perilesional edema also resolved faster in albendazole group (p<0.05). Thus, albendazole did not alter the long-term seizure outcome in patients with SCCL and epilepsy. However, albendazole hastened resolution of SCCL on MRI, but interestingly 23% of lesions were still active 12 months after treatment.

T2 relaxometry helps prognosticate seizure outcome in patients with solitary cerebral cysticercosis.

OBJECTIVE: Correlate serial T2 relaxometry (T2R) values with long term seizure outcome in patients with solitary cerebral cysticercosis (SCC) in order to establish its usefulness as a prognostic marker in these patients. METHODS: Patients with new-onset seizures due to SCC were imaged serially using a pre-determined MRI protocol at enrolment and after 3, 6, 12 and 24months. T2 relaxometry was performed using a dual echo sequence with maps generated manually from the measured image intensities at the level of the lesion. Patients were randomised to receive albendazole plus antiepileptic drugs, or only antiepileptic treatment ("controls"). At each visit, as well as four years after study initiation, patients were reviewed for seizure recurrence. Clinical and radiological outcomes were assessed by physicians blinded to treatment received. RESULTS: Of 123 patients recruited, 77 had at least four MRIs and >12month follow-up, and were included for analysis. Baseline clinical and demographic parameters as well as antiepileptic treatment were similar between albendazole and control groups. T2 values from the lesion were higher than normal parenchyma initially, and fell to approach normal over six months. Controls had higher T2 values from the lesion centre and wall at six months than those who received albendazole. However no difference was seen in T2 values from perilesional parenchyma between treatment and control groups, indicating lack of modulation of the development of perilesional gliosis by albendazole therapy. Patients with seizures persisting >6months after enrolment had higher perilesional T2 values than those who were seizure-free. A rise in perilesional T2 value at 12months is probably due to gliosis. A later stage of degeneration was associated with a reduced likelihood of seizure relapse. SIGNIFICANCE: T2 relaxometry at three and six months after seizure onset can identify patients likely to have seizures beyond six months after onset. Persistently abnormal T2 values in patients with poorer outcomes reflect the development of perilesional gliosis.

Acute multifocal neuropathy following cocaine inhalation.

INTRODUCTION: We report a young man, not a habitual cocaine user, who developed an acute multifocal neuropathy following a second exposure to inhaled cocaine. METHODS: Case report. RESULTS: Clinical and electrophysiological findings suggested an acute multiple mononeuropathy following cocaine exposure. Imaging of the shoulder and pelvic girdles revealed multifocal denervation in selected proximal muscles. The patient was empirically treated with intravenous steroids to good effect. DISCUSSION: Cocaine use, although usually affecting the central nervous system, does produce peripheral nerve disease in rare instances. This unusual pattern of neurological involvement needs to be differentiated from the more common symptoms resulting from affection of the brain.

Delayed-onset Reversible Cortical Blindness after Resuscitation from Cardiac Arrest.

We present a patient who presented with cortical blindness (CB) 1 week after repeated cardiac arrest while undergoing treatment for an acute myocardial infarction. He had been revived within 5 min in each instance and was apparently neurologically normal until presentation. Magnetic resonance imaging showed subtle hyperintensities on fluid-attenuated inversion recovery and diffusion-weighted imaging in both temporooccipital cortices. A rapid recovery over the next 2 weeks was remarkable for the appearance of metamorphopsia. CB may present even days to weeks after hypoxic-ischemic encephalopathy following cardiac arrest, even in patients apparently without immediate neurological sequelae. The pathogenesis of this phenomenon remains to be fully elucidated, but is likely to be due to delayed effects of anoxia on the occipital cortex and may be analogous to the previously described syndrome of delayed posthypoxic leukoencephalopathy. Prognosis for visual recovery appears to be good.