Wenke Grönheit

Impairment of Vowel Articulation as a Possible Marker of Disease Progression in Parkinson's Disease

Purpose The aim of the current study was to survey if vowel articulation in speakers with Parkinsons disease (PD) shows specific changes in the course of the disease. Method 67 patients with PD (42 male) and 40 healthy speakers (20 male) were tested and retested after an average time interval of 34 months. Participants had to read a given text as source for subsequent calculation of the triangular vowel space area (tVSA) and vowel articulation index (VAI). Measurement of tVSA and VAI were based upon analysis of the first and second formant of the vowels /α/, /i/and /u/ extracted from defined words within the text. Results At first visit, VAI values were reduced in male and female PD patients as compared to the control group, and showed a further decrease at the second visit. Only in female Parkinsonian speakers, VAI was correlated to overall speech impairment based upon perceptual impression. VAI and tVSA were correlated to gait impairment, but no correlations were seen between VAI and global motor impairment or overall disease duration. tVSA showed a similar reduction in the PD as compared to the control group and was also found to further decline between first and second examination in female, but not in male speakers with PD. Conclusions Measurement of VAI seems to be superior to tVSA in the description of impaired vowel articulation and its further decline in the course of the disease in PD. Since impairment of vowel articulation was found to be independent from global motor function but correlated to gait dysfunction, measurement of vowel articulation might have a potential to serve as a marker of axial disease progression.

Effect of Subthalamic Stimulation on Voice and Speech in Parkinson’s Disease: For the Better or Worse?

Background: Deep brain stimulation of the subthalamic nucleus, although highly effective for the treatment of motor impairment in Parkinson’s disease (PD), can induce speech deterioration in a subgroup of patients. The aim of the current study was to survey (1) if there are distinctive stimulation effects on the different parameters of voice and speech and (2) if there is a special pattern of preexisting speech abnormalities indicating a risk for further worsening under stimulation. Methods: N = 38 patients with PD had to perform a speech test without medication with stimulation ON (StimON) and stimulation OFF (StimOFF). Speech samples were analyzed: (1) according to a four-dimensional perceptual speech score and (2) by acoustic analysis to obtain quantifiable measures of distinctive speech parameters. Results: Quality of voice was ameliorated with StimON, and there were trends of increased loudness and better pitch variability. N = 8 patients featured a deterioration of speech with StimON, caused by worsening of articulation or/and fluency. These patients already had more severe overall speech impairment with characteristic features of articulatory slurring and articulatory acceleration under StimOFF condition. Conclusion: The influence of subthalamic StimON Parkinsonian speech differs considerably between individual patients, however, there is a trend to amelioration of voice quality and prosody. Patients with stimulation-associated speech deterioration featured higher overall speech impairment and showed a distinctive pattern of articulatory abnormalities at baseline. Further investigations to confirm these preliminary findings are necessary to allow neurologists to pre-surgically estimate the individual risk of deterioration of speech under stimulation.

A systematic review of suggestive seizure induction for the diagnosis of psychogenic nonepileptic seizures

Suggestive seizure induction is a widely used method for diagnosing psychogenic nonepileptic seizures (PNES). Despite seven decades of multidisciplinary research, however, there is still no unified protocol, no definitive agreement on the ethical framework and no consensus on diagnostic utility. This systematic review surveys the evidence at hand and addresses clinically relevant aspects of suggestive seizure induction. In addition to its use for facilitating the diagnostic process, its mechanism of action and utility in elucidating the psychopathology of PNES will be discussed.

Hyperventilation and photic stimulation are useful additions to a placebo-based suggestive seizure induction protocol in patients with psychogenic nonepileptic seizures

The early and definitive diagnosis of psychogenic nonepileptic seizures is a common challenge in epileptology practice. Suggestive seizure induction is a valuable tool to aid the differentiation between epileptic and psychogenic nonepileptic seizures, especially when long-term video-EEG monitoring is inconclusive or unavailable. In this retrospective analysis, we compared the diagnostic yield of a classical, placebo-based induction protocol with that of an extended protocol that includes hyperventilation and photic stimulation as means of suggestion while also implementing more open, standardized patient information. We investigated whether the diversification of suggestive seizure induction has an effect on diagnostic yield and whether it preempts the administration of placebo. Data from 52 patients with confirmed psychogenic nonepileptic seizures were analyzed. While suggestive seizure induction using only placebo-based suggestion provoked a typical event in 13 of 20 patients (65%), the extended protocol was positive in 27 of 34 cases (84%); this improvement was not significant (p=0.11). Noninvasive suggestion techniques accounted for 78% of inductions, avoiding placebo administration in a majority of patients. Still, placebo remains an important part of suggestive seizure induction, responsible for 22% (6 out of 27) of successful inductions using our extended protocol. Our study demonstrates that the diversification of suggestive seizure induction is feasible and beneficial for both patients and diagnosticians.

Instability of syllable repetition in progressive supranuclear palsy

Dysarthria is a prominent feature of progressive supranuclear palsy (PSP) and consists—amongst other features—of impaired speech fluency. Since in Parkinson’s disease (PD) steadiness of syllable repetition in the course of the performance has been shown to be impaired, the aim of the present study was to investigate if measurement of syllable repetition shows similar or even more pronounced abnormalities in PSP. Thirty six patients with the clinical diagnosis of PSP (16 PSP-Richardson syndrome/PSP-RS and 20 PSP-parkinsonism/PSP-P), 60 patients with PD and 32 healthy speakers were tested. Participants had to repeat a single syllable in a self chosen isochronous pace. Percental coefficient of variance (COV) of interval length was measured for description of pace stability throughout the performance. All patients were tested according to Unified Parkinson’s Disease Rating Scale (UPDRS III). Patients with PSP were additionally scored according to PSP rating scale (PSPRS). In the PSP group, COV was significantly worse not only when compared with healthy speakers but also when compared with PD patients of similar disease duration and similar global motor impairment. COV showed only a trend to higher values in the subgroup with PSP-RS as compared to PSP-P. In the PSP-P, but not in the PSP-RS subgroup, there was a positive correlation between COV and PSPRS/UPDRS III. PSP patients feature more pronounced difficulties in the steady syllable repetition than patients with PD and healthy controls as a hint for a more profound dysfunction of basal motor speech performance in PSP than in PD.

Two different phenomena in basic motor speech performance in premanifest Huntington disease

Objective: Dysarthria is a common feature in Huntington disease (HD). The aim of this cross-sectional pilot study was the description and objective analysis of different speech parameters with special emphasis on the aspect of speech timing of connected speech and nonspeech verbal utterances in premanifest HD (preHD). Methods: A total of 28 preHD mutation carriers and 28 age- and sex-matched healthy speakers had to perform a reading task and several syllable repetition tasks. Results of computerized acoustic analysis of different variables for the measurement of speech rate and regularity were correlated with clinical measures and MRI-based brain atrophy assessment by voxel-based morphometry. Results: An impaired capacity to steadily repeat single syllables with higher variations in preHD compared to healthy controls was found (variance 1: Cohen d = 1.46). Notably, speech rate was increased compared to controls and showed correlations to the volume of certain brain areas known to be involved in the sensory-motor speech networks (net speech rate: Cohen d = 1.19). Furthermore, speech rate showed correlations to disease burden score, probability of disease onset, the estimated years to onset, and clinical measures like the cognitive score. Conclusions: Measurement of speech rate and regularity might be helpful additional tools for the monitoring of subclinical functional disability in preHD. As one of the possible causes for higher performance in preHD, we discuss huntingtin-dependent temporarily advantageous development processes of the brain.

Faciobrachio-crural dystonic seizures in LGI1 limbic encephalitis: A treatable cause of falls

Faciobrachial dystonic seizures (FBDS) are closely associated with antibodies (Ab) to the leucine-rich glioma inactivated-1 protein (LGI1) component of the voltage-gated potassium channel (VGKC) complex. They precede or indicate the onset of limbic encephalitis (LE).1 Immunotherapy can prevent the development of LE and cognitive impairment.1,2

Recurrent loss of consciousness despite DDD pacing: psychogenic pseudosyncope in a 19-year-old man.

Unexplained recurrent syncope requires interdisciplinary assessment. Paroxysmal bradyand tachycardias can mimic psychiatric conditions [1, 2] and, on the other hand, epileptic seizures, panic attacks and psychogenic phenomena can be mistaken for dysrhythmias. While the former misdiagnosis can be avoided by structured cardiological workup, the latter requires neurological assessment considering the differential diagnosis psychogenic pseudosyncope [3]. A 19-year-old man with recurrent syncope of unknown origin was referred to our epilepsy center for neurological assessment. Attacks with transient loss of consciousness first occurred 2 years previously. A typical event would often be preceded by a rising sensation of pressure and pulsation in the neck and head, followed by a (usually backwards) fall. The ensuing unconsciousness usually lasted 4–5 min with reorientation afterwards lasting several minutes. No motor activity, tongue bite or enuresis during the events was reported. Within 3 months prior to neurological assessment, 1–2 events per week were noted. Previous medical history included autonomic dysfunction, arterial hypertension, nicotine addiction, obesity (135 kg) and obstructive sleep apnea; he was diagnosed in other institutions as having ‘‘Tourette’s syndrome, social phobia, Asperger syndrome’’ and ‘‘attention deficit disorder’’. Multiple cardiological investigations had led to implantation of an event recorder, which registered eventcorrelated narrow complex tachycardias of up to 187 bpm as well as bradycardias of 40 bpm. An electrophysiological study revealed no sign of habitual arrhythmia and no inducible tachycardias. ‘‘Sick sinus syndrome’’ was diagnosed and a DDD pacemaker was implanted. However, the attacks persisted with unaltered frequency. On repeated cardiological assessment, pacemaker function was intact and no signs of autonomic dysfunction or dysrhythmia were found. A neurological investigation was recommended. During 2 days of continuous electroencephalography(EEG-)video monitoring, no interictal epileptic discharges were recorded and no transient loss of consciousness occurred. Tilt-table test was impossible due to weight restrictions of the equipment; Schellong testing was normal with no sign of autonomic dysfunction. The patient was then subjected to suggestive ‘‘seizure’’ provocation through postural maneuvers (sitting down and standing up swiftly) and intravenous administration of saline (mixed with Vitamin B12 for pink coloring); he was informed that these techniques induced fits in some patients. Mattresses were placed on the floor to avoid injury. Rapid postural changes akin to Schellong testing did not elicit loss of consciousness or any significant changes in blood pressure. After three consecutive injections, however, the patient suddenly fell to the ground and was unresponsive for 92 s. Before, during and after the event Electronic supplementary material The online version of this article (doi:10.1007/s00392-014-0711-5) contains supplementary material, which is available to authorized users.

Practical Management of Epileptic Seizures and Status Epilepticus in Adult Palliative Care Patients

In terminally ill patients, paroxysmal or episodic changes of consciousness, movements and behavior are frequent. Due to ambiguous appearance, the correct diagnosis of epileptic seizures (ES) and non-epileptic events (NEE) is often difficult. Treatment is frequently complicated by the underlying condition, and an approach indicated in healthier patients may not always be appropriate in the palliative care setting. This article provides recommendations for diagnosis of ES and NEE and treatment options for ES in adult palliative care patients, including aspects of alternative administration routes for antiepileptic drugs such as intranasal, subcutaneous, or rectal application.

Progressive hippocampal sclerosis after viral encephalitis: Potential role of NMDA receptor antibodies.

PURPOSE Survivors of viral encephalitis can develop refractory epilepsy and hippocampal sclerosis. Both the initial infectious insult and the secondary effects of recurrent seizures have been implicated in chronic disease progression. Recently, post-infectious autoimmunity, involved in acute relapses, has also been proposed as a pathomechanism for chronic disease progression. Our case series suggests a potential role of antibodies against the N-methyl-d-aspartate receptor (NMDAR) in chronic inflammatory disease beyond acute manifestations. METHODS Retrospective chart review of four patients with epilepsy, hippocampal sclerosis following viral encephalitis and NMDAR-antibodies in CSF. RESULTS The four patients were female, developed hippocampal sclerosis (in 3/4 in a step-wise progression) after Herpes simplex or Varicella zoster virus encephalitis and harboured immunoglobulin G antibodies against the NMDAR in their CSF. Two patients were treated with short-term immunosuppression but did not benefit. CONCLUSION This case series presents the first tentative evidence in support of chronic autoimmune inflammation driving disease progression after viral encephalitis beyond the known acute immune-mediated relapses. The anecdotal nature of the data does not, however, permit conclusive judgement on causality. Should our findings be replicated in larger cohorts, the treatment of post-infectious epilepsy could potentially be expanded to include immunosuppressive strategies in antibody-positive cases.