IMPORTANCE-Functional neurological disorders (FND) are common sources of disability in medicine. Patients have often been misdiagnosed, correctly diagnosed after lengthy delays, and/or subjected to poorly delivered diagnoses that prevent diagnostic understanding and lead to inappropriate treatments, iatrogenic harm, unnecessary and costly evaluations, and poor outcomes. OBSERVATIONS-Functional Neurological Symptom Disorder/Conversion Disorder was adopted by the Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition, replacing the term psychogenic with functional and removing the criterion of psychological stress as a Funding/Support: This article presents independent research part-funded by the

The clinical and laboratory phenotype of a paraproteinaemic neuropathy syndrome termed chronic sensory ataxic neuropathy with anti-disialosyl IgM antibodies is described in a series of 18 cases. Previous single case reports have outlined some features of this syndrome. All 18 cases were defined by the presence of serum IgM antibodies which react principally with NeuAc (alpha2-8)NeuAc(alpha2-3)Gal-configured disialosyl epitopes common to many gangliosides including GDlb, GD3, GTlb and GQlb. In 17 out of 18 cases, the serum contained benign IgM paraproteins, and in four of these cases at least two IgM paraproteins were present. The IgM antibodies were also cold agglutinins in 50% of cases. The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs. In addition, 16 out of 18 cases had motor weakness affecting oculomotor and bulbar muscles as fixed or as relapsing-remitting features. When present in their entirety, these clinical features have been described previously under the acronym CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM paraprotein, cold agglutinins and disialosyl antibodies. This distribution of clinical features is reminiscent of Miller Fisher syndrome, in which acute-phase anti-disialylated ganglioside IgG antibodies are found. Clinical electrophysiology and nerve biopsy show both demyelinating and axonal features. A partial response to intravenous immunoglobulin and other treatments is reported in some cases.

Objectives: Patients with intractable epilepsy may benefit from epilepsy surgery especially if they have a radiologically demonstrable cerebral lesion. Dedicated magnetic resonance imaging (MRI) protocols as performed at epilepsy surgery centres can detect epileptogenic abnormalities with great sensitivity and specificity. However, many patients with epilepsy are investigated with standard MRI sequences by radiologist outside epilepsy centres ("non-experts"). This study was undertaken to compare standard MRI and epilepsy specific MRI findings in patients with focal epilepsy. Methods: Comparison of results of standard MRI reported by "non-expert" radiologists, standard MRI evaluated by epilepsy "expert" radiologists, and epilepsy specific MRI read by "expert" radiologists in 123 consecutive patients undergoing epilepsy surgery evaluation between 1996 and 1999. Validation of radiological findings by correlation with postoperative histological examination. Results: Sensitivity of "non-expert" reports of standard MRI reports for focal lesions was 39%, of "expert" reports of standard MRI 50%, and of epilepsy dedicated MRI 91%. Dedicated MRI showed focal lesions in 85% of patients with "non-lesional" standard MRI. The technical quality of standard MRI improved during the study period, but "non-expert" reporting did not. In particular, hippocampal sclerosis was missed in 86% of cases. Neuropathological diagnoses (n=90) were predicted correctly in 22% of "non-expert" standard MRI reports but by 89% of dedicated MRI reports. Conclusions: Standard MRI failed to detect 57% of focal epileptogenic lesions. Patients without MRI lesion are less likely to be considered candidates for epilepsy surgery. Patients with refractory epilepsy should be referred to an MRI unit with epileptological experience at an early point.T he prevalence of epilepsy is 0.5%-1%.1 About 75% of patients with epilepsy can be treated satisfactorily with antiepileptic drugs. However, treated success depends on epilepsy syndrome and seizure types. For example, antiepileptic drugs fail to control seizures in up to 75% of localisation related epilepsies.2-5 For medically intractable epilepsy patients, surgical treatment is the major therapeutic alternative. About 70% of patients who undergo epilepsy surgery become seizure free. The success rate may be even higher in patients with medial temporal lobe epilepsy. As a result of the constant improvement of image quality, magnetic resonance imaging (MRI) has become one of the most important tools in the selection of patients for epilepsy surgery.8 Congruity of a radiologically demonstrable lesion, ictal epileptic discharges in the EEG, and seizure semiology may permit epilepsy surgery without further invasive diagnostic procedures. In patients in whom scalp EEG recordings are insufficient, MRI abnormalities may generate a hypothesis for intracranial electrode implantation. 6 Often, MRI is performed repeatedly during the course of a seizure disorder. It may be undertaken to aid the initial diagnostic process and r...

SUMMARYThe International League Against Epilepsy (ILAE) Neuropsychobiology Commission gave the charge to provide practical guidance for health professionals for the pharmacologic and nonpharmacologic treatment of patients with psychogenic nonepileptic seizures (PNES). Using a consensus review of the literature, an international group of clinician-researchers in epilepsy, neurology, neuropsychology, and neuropsychiatry evaluated key management approaches for PNES. These included the following: presentation of the diagnosis, early phase treatment, psychological and pharmacologic interventions, and maintenance management. The aim of this report is to provide greater clarity about the range and current evidence base for treatment for patients with PNES, with the intention of improving the care of patients with PNES and patients who develop PNES as a comorbidity of epilepsy.