Paroxysmal dysarthria-ataxia syndrome.

Paroxysmal dysarthria ataxia syndrome presents with recurrent, brief, stereotyped events of dysarthria, limb clumsiness, unsteady gait and vertigo or dizziness that can occur in association with lesions in the midbrain. We describe a case of a woman presenting with paroxysmal dysarthria and ataxia secondary to a midbrain lesion, treated successfully with carbamazepine.

MRI-verified "asleep" deep brain stimulation in Malta through cross border collaboration: clinical outcome of the first five years.

INTRODUCTION: Deep Brain Stimulation (DBS) requires a specialist multidisciplinary approach and lifelong follow-up. Patient access can be a challenge for small nation states. Malta is an island nation with a population of just under 450 000. The number of patients likely to benefit from DBS is around 5 to 10 per year. This study explores the outcome of a cross border collaboration between specialist services at Queen Square, London and a tertiary centre in Malta. MATERIAL AND METHODS: Between 2011 and 2015, 35 patients underwent MRI-Guided and MRI-Verified DBS with 29 receiving bilateral subthalamic nucleus (STN) DBS for Parkinson's Disease under general anaesthesia. Pre-operative motor function was compared with one year post-operative motor function assessments in 26 patients (16 male; age 60 ± 9, range 32-70; disease duration 8.8 ± 2.7). Pre-operative and post-operative quality of life scores were also completed in 24 patients. RESULTS: There was significant improvement in off-medication Unified Parkinson's Disease Rating Scale (UPDRS) III motor function (41.7%), reduction in Levodopa Equivalent Dose (LED) (30.6%) and improvement in quality of life as measured by the Parkinson's Disease Questionnaire (PDQ-39) (52.3%) (p < .001). All PDQ-39 dimensions showed significant improvement except communication, with greatest benefit in activities of daily living (ADLs) (72.4%) and stigma (66.3%). Surgical complications did not lead to any permanent deficit. Patients receiving DBS to other targets and for different indications also benefitted from surgery. CONCLUSION: An MRI-guided and MRI-verified approach to DBS was successfully implemented through cross border collaboration with achievement of expected clinical results. This healthcare collaboration developed out of necessity and opportunity, taking advantage of a UK-based neurosurgeon from Malta. The UK healthcare system benefits from numerous immigrants at Consultant level. Such a mutually beneficial arrangement could enable such individuals to offer their expertise to citizens in the UK as well as their country of origin.

Encephalitis as an initial presentation of type 2 amiodarone-induced thyrotoxicosis.

We report a case of a woman in her mid-20s presenting with encephalitis as the initial presentation of type 2 amiodarone-induced thyrotoxicosis (AIT). She was on amiodarone in view of a history of hypertrophic cardiomyopathy. Symptomatology included acute personality change and focal myoclonic jerks.Cerebrospinal fluid analysis showed a non-specific protein count elevation with negative microbiology, virology, autoimmune screen and onconeural antibodies. The electroencephalogram was consistent with a generalised cerebral dysrhythmia. An MRI of the head revealed symmetrical oedema within the motor cortices and a high T2 signal within the cerebellar dentate nuclei, with no restricted diffusion. Blood investigations confirmed thyrotoxicosis with negative antithyroid antibodies. She did not fulfil the criteria for a thyroid storm. Other possible causes of encephalitis were excluded.There was an excellent clinical, laboratory and radiological response to glucocorticoids, suggesting a diagnosis of steroid-responsive encephalitis secondary to type 2-AIT in the absence of a thyroid storm.

Postintervention acute opsoclonus myoclonus syndrome.

Opsoclonus myoclonus syndrome (OMS) is a rare, neurological condition affecting 1 in 10 000 000 people annually. Opsoclonus, defined as involuntary rapid, multivectorial oscillations of the eyes, together with ataxia and myoclonus are usually present. OMS may be paraneoplastic: often associated with occult neuroblastoma in childhood and with breast carcinoma or small cell lung carcinoma in adults. Other aetiologies include viral or toxic agents. The pathogenesis is thought to be immune mediated. A 37-year-old woman with previous inflammatory cranial mononeuropathies was admitted for elective dilatation and curettage (D&C). Immediately after she complained of left-sided paraesthesia and later became disoriented, with incoherent speech, inability to obey commands, opsoclonus of the eyes and myoclonic jerks. Investigations including onconeuronal antibodies, cerebrospinal fluid analysis, and imaging were normal. She was treated with intravenous methylprednisolone with rapid improvement. Previous surgeries with anaesthesia were uncomplicated. The anaesthetic agents used for the D&C were fentanyl and propofol.