C. Lechner

Twenty-four-hour blood pressure profile and blood pressure responses to head-up tilt tests in Parkinson's disease and multiple system atrophy.

Objective To investigate the 24 h blood pressure profile in patients with Parkinsons disease with intact autonomic function or with autonomic failure and patients with multiple system atrophy (MSA), and to assess whether these patients exhibit posture-related variations in blood pressure. Patients and methods We studied 24 patients with Parkinsons disease (11 with autonomic failure) and 13 patients with MSA (all with autonomic failure). Autonomic failure was determined by autonomic tests. An oscillometric recorder was used for ambulatory blood pressure monitoring. Tilt-table tests were performed with a head-up tilt position of 60°. Results An alteration in the normal 24 h blood pressure profile was observed in 82% of Parkinsons disease patients with autonomic failure and in 85% of those with multiple system atrophy, but not in the patients with intact autonomic function. Head-up tilt tests revealed a significantly higher supine blood pressure in Parkinsons disease patients with autonomic failure and in those with MSA than in Parkinsons disease patients with intact autonomic function. Tilting resulted in a marked fall in blood pressure in patients with MSA; in Parkinsons disease patients with autonomic failure, the fall was comparatively slighter. Conclusions We conclude that autonomic failure contributes to the alterations in the day–night blood pressure profile that may possibly be ascribed to postural dysregulation of blood pressure. We hypothesize that nocturnal hypertension is a risk factor in the development of additional cerebrovascular disease in patients with Parkinsons disease or MSA who are affected by autonomic failure.

Orienting response and frontal midline theta activity: a somatosensory spectral perturbation study

OBJECTIVE Brain electric activity in the theta frequency band has been associated with the encoding of new, and the retrieving of previously stored, information. We studied the time course of stimulus-to-stimulus changes of theta activity under repetitive somatosensory stimulation. MATERIALS AND METHODS Twelve healthy subjects participated in the study. Repetitive electric stimuli, grouped into 48 stimulus trains, were applied to the left index finger. The stimulus trains contained 27 stimuli (0.9 Hz, 2.5 times sensory threshold). Each stimulus train of 30 s was followed by a stimulus-free break of 30 s. This stimulation paradigm allowed the separate estimation of effects for each position of the stimulus in the train and an analysis of stimulus-to-stimulus changes. Multichannel EEG recordings allowed a topographic analysis of the event-related spectral perturbation effects in the theta frequency band. The brain electric novelty response triggered by the stimulus train onset was analyzed by 3 methods: (1) event-related potentials; (2) event-related power spectra for the investigation of spectral perturbation effects on theta activity; and (3) an approach to break down the stimulus-induced theta activity into phase-locked activity and effects on the spontaneous, ongoing theta activity using digital filtering. RESULTS The main findings are a frontal midline activation in the theta band with the beginning of the stimulus train, which habituates during the subsequent stimulation cycles, as well as evidence that distinct effects of the first stimulus on the ongoing, non-phase-locked, theta activity exist.

Magnetic resonance imaging findings in corticobasal degeneration.

Two women (patient 1, 77 years old, and patient 2, 63 years old) with strong clinical evidence for corticobasal degeneration (CBD) are presented. Patient 2 was in an early stage of the disease with only a mild disability of her left hand. In addition to the clinical characteristics, both patients presented the typical cortical reflex myoclonus. Magnetic resonance imaging studies for both patients revealed nearly identical hyperintense lesions somatotopic from the left‐hand primary motor cortex (M1), extending to the midline and possibly supplementary motor area (SMA) in patient 2. To our knowledge, this has not been previously described in patients with CBD. These lesions may play a role in the etiology and the development of CBD with involvement of the M1 and may correspond to the underlying pathology of demyelination or gliosis.

Acute and chronic neuroendocrine effects of interferon-beta 1a in multiple sclerosis.

Objective  Treatment of multiple sclerosis with interferon‐β (IFN‐β) results in variable responses interindividually. Cytokine–hormone interactions may modulate the therapeutic effects of IFN‐β. Since hyperactivity of the hypothalamo‐pituitary‐adrenal (HPA) axis and other neuroendorine disturbances occur in multiple sclerosis, we determined the detailed neuroendocrine response of patients with multiple sclerosis to IFN‐β.

Non‐convulsive status epilepticus with marked neuropsychiatric manifestations and MRI changes after treatment of hypercalcaemia

We describe a 77‐year‐old woman who developed a confusional state, cognitive impairment, behavioural abnormalities and dysphasia after treatment of hypercalcaemia. Repeated EEG recording revealed rhythmic sharp‐wave activity over the right parietal‐occipital lobe. Magnetic resonance imaging (MRI) showed marked hyperintense signal changes bilaterally. The diagnosis of a non‐convulsive status epilepticus (NCSE) was made. With antiepileptic treatment the patient improved and MRI as well as EEG changes were almost all reversible. NCSE is an important differential diagnosis of patients with neuropsychiatric symptoms and can develop after rapid lowering of serum calcium levels in hypercalcaemia.

Enhanced long-latency somatosensory potentials in major depressive disorder

Bodily misperceptions are a frequent symptom in major depressive disorder. A reduced ability to deflect attention from somatosensory stimuli may contribute to the generation of unpleasant bodily sensations and co-occur with altered habituation of the brain electric reactions to somatosensory stimuli. The aim of the present study was to explore whether attention-related components of somatosensory evoked potentials (SSEP) and the habituation of these components are altered in major depression. Fifteen patients with major depressive disorder were compared to an age- and gender-matched group of 15 healthy controls. A series of identical, intrusive but not painful electric stimuli were applied to the left index finger for 48 min. Averaged SSEP were computed from multichannel EEG recordings for consecutive recording blocks of the experiment, each block containing 162 stimuli. Based on these data the habituation process of late components of the SSEP was analysed in two latency intervals (50-150, 170-370 ms). Patients showed significantly enhanced reactions throughout the entire experiment. The persistence of enhanced SSEP components throughout the habituation process may be caused by a deficit in reducing the activity of attention-related brain processes concerned with intrusive, yet behaviourally irrelevant, continued stimulation in the state of major depression.

Involuntary vocalisations and a complex hyperkinetic movement disorder following left side thalamic haemorrhage

A variety of involuntary speech phenomena as for example palilalia have been described as consequences of neurological disorders. Palilalia is the involuntary repetition of syllabels, words and phrases in ongoing speech. We describe a 73 year old woman who suffered from a hypertensive thalamic haemorrhage. MRI revealed that the lesion was predominantly located within the pulvinar, extending to the lateroposterior thalamic nuclei and to the pretectal area with possible involvement of the medial geniculate body. Few months after the event she developed involuntary vocalisations with whole words and meaningless syllables being rapidly reiterated. In contrast to typical palilalia these vocalisations were not meaningfully related to the ongoing speech of the patient. In addition, the patient developed a complex hyperkinetic movement disorder with right-sided painful hemidystonia and bilateral clonic jerks and a right-sided postural tremor.

Exazerbation von Tics durch Prednisolon

ZusammenfassungTics sind eine Form von hyperkinetischer Bewegungsstörung, die sich üblicherweise in der Kindheit oder in der frühen Adoleszenz manifestiert und im späteren Erwachsenenalter an Intensität verliert. Wir berichten über die erste klinisch relevante Exazerbation einer Ticstörung bei 2 Patientinnen mit einer bis zum 53. bzw. 68. Lebensjahr subklinisch verlaufenden Symptomatik. Während der Prednisolontherapie einer Polymyalgia rheumatica bzw. einer endokrinen Orbitopathie bei M. Basedow exazerbierten vorbestehende subklinische Tics, so daß die Ticstörung diagnostiziert und zum erstenmal pharmakologisch behandelt wurde. Mögliche auslösende Faktoren für Tics während einer Therapie mit Glukokortikoiden werden im Kontext der Dopaminhypothese von Tics diskutiert.SummaryTics-disorders are hyperkinetic movement disorders which manifest in childhood or early adolescence and ameliorate in later adulthood. We report on two patients with a basically subclinical tic-disorder (age of 53 and 68 years). During the therapy of polymyalgia rheumatica and endocrine orbitopathy in Graves disease with prednisolone the tics exacerbated and were therefore diagnosed and treated for the first time. Possible causes for the exacerbation of the tics during the therapy with glucocorticosteroids are discussed in the context of the dopamine hypothesis of tics.