Guillain-Barré Syndrome and Its Variants: Clinical Course and Prognostic Factors.

INTRODUCTION: We aimed to analyze the frequency, clinical characteristics, medical treatment options and final functional status of Guillain-Barré syndrome (GBS) and its variants in a population from a tertiary hospital setting. METHODS: All medical records of patients with acute inflammatory polyneuropathy between the years of 1998-2013 were retrospectively screened. Demographic, clinical and laboratory information, treatment options and the rate of recovery of the patients were gathered. RESULTS: A total of 183 patients met the study criteria. Subtypes were typical demyelinating form (n=102, 79.1%), acute motor sensory axonal variant (n=11, 8.5%), acute motor axonal variant (n=10, 7.8%), Miller-Fisher syndrome (n=5, 3.9%), and pure sensory subtype (n=1, 0.8%). Remaining patients had the diagnosis of acute-onset chronic inflammatory demyelinating polynuropathy. The data of treatment option were available for 70 patients. Most of the patients received intravenous immunoglobulin (IVIg) treatment or the combination of IVIg and methylprednisolone. One patient died, there was no improvement in eight patients and rest showed improvement with varying degrees. CONCLUSIONS: We did not observe major change of recovery between different treatment options, however, most of the patients using methylprednisolone required IVIg because of inadequate response.

Re-emergent Tongue Tremor as the Presenting Symptom of Parkinson's Disease.

BACKGROUND: The most common symptom of Parkinson's disease is the unilateral, typically resting tremor in body parts, most commonly in the upper extremities. However, this finding can spread to the other parts of the body like lips, chin, jaw and tongue during the course of the disease. Nevertheless, we have not come across any Parkinson's disease case presenting with tongue tremor in the literature. CASE REPORT: Here, we present a 58 year-old man with Parkinson's disease presenting with tongue tremor, his striking response to the levodopa test and his follow-up data. CONCLUSION: The topography of motor symptoms at onset in Parkinson's disease is presumably determined by the severity of the Lewy body neuronal degeneration in distinct parts of the substantia nigra. Therefore, patterns of somatic symptom progression in Parkinson's disease indicate that involvement of the cranial structures always follows the development of symptoms in the extremities. However, in our case, cranial structures seem to precede the involvement of extremity-related areas.

A close look at EEG in subacute sclerosing panencephalitis.

PURPOSE: To define atypical clinical and EEG features of patients with subacute sclerosing panencephalitis that may require an overview of differential diagnosis. METHODS: A total of 66 EEGs belonging to 53 (17 females and 36 males) consecutive patients with serologically confirmed subacute sclerosing panencephalitis were included in this study. Patient files and EEG data were evaluated retrospectively. EEGs included in the study were sleep-waking EEGs and/or sleep-waking video-EEG records with at least 2 hours duration. Cranial MRIs of the patients taken 2 months before or after the EEG records were included. RESULTS: Age range at the onset of the disease was 15 to 192 months (mean age: 80.02 months). Epilepsy was diagnosed in 21 (43%) patients. Among epileptic seizures excluding myoclonic jerks, generalized tonic-clonic type constituted the majority (58%). Tonic seizures were documented during the video-EEG recordings in four patients. Epileptogenic activities were found in 56 (83%) EEG recordings. They were localized mainly in frontal (58%), posterior temporal, parietal, occipital (26%), and centrotemporal (8%) regions. Multiple foci were detected in 26 recordings (39%). Epileptiform activities in the 39 (59%) EEGs appeared as unilateral or bilateral diffuse paroxysmal discharges. CONCLUSIONS: Recognition of uncommon clinical and EEG findings of subacute sclerosing panencephalitis, especially in countries where subacute sclerosing panencephalitis has not been eliminated yet, could be helpful in prevention of misdiagnosis and delay in the management of improvable conditions.

End of the barbexaclone era: an experience of treatment withdrawal.

Barbexaclone is a salt compound of phenobarbital and propylhexedrine (a drug with indirect sympathomimetic properties). Due to the presence of the psychostimulating agent, propylhexedrine, this drug has less of a sedative effect and is well tolerated, compared to phenobarbital. Barbexaclone was widely used in Turkey until 2009 when its production ended, however, it gave rise to an epidemic for which we were not prepared. Since then, no standardised management protocol has been developed and each patient has been evaluated individually, thereby creating tailor-made solutions based on the extent of each patient's supply of remaining drug (from a few tablets to a stock which might last for six months). The rate of seizure freedom was 37.7% under barbexaclone treatment and dropped to 32.2% in the follow-up period after discontinuation of the drug. In the majority of cases, a new antiepileptic drug was added and this was commonly levetiracetam, a more expensive drug. In this article, we share our experiences of a general problem: the withdrawal of an antiepileptic drug from the market. Although there was prior notification regarding barbexaclone withdrawal, it was not possible to contact all patients since such a database is not available in Turkey. Although no conclusions regarding the efficacy of the drug or comparison of efficacy with other antiepileptic drugs is provided, it is nonetheless noteworthy to share these experiences since some patients had lost seizure control for reasons that could not be explained.

A case imitating Panayiotopoulos syndrome.

Panayiotopoulos syndrome is one of the most common childhood-specific epileptic disorders. It is characterized by autonomic symptoms; the most common being emesis. However, the presence of emetic symptoms may cause misdiagnosis, for example with some organic disorders which have similar autonomic features. On the other hand, since this syndrome has been recently well documented, the tendency for early recognition may lead to overdiagnosis. Here, we present a case which could be mistaken for Panayiotopoulos syndrome based on anamnesis, however, with the aid of ictal video-EEG monitoring, the patient was shown to present with a non-epileptic psychogenic seizure. This report is an example of careful evaluation in order to avoid over- or underdiagnosis of this benign disorder. [Published with video sequences].