Post-ischemic Holmes’ tremor of the lower extremities

Holmes' tremor, which is known to occur as a result of different lesions centered in the brain stem, cerebellum and thalamus, is a tremor of low frequency, mostly below 4.5 Hz (Deuschl and Bergman in Mov Disord 17(suppl 3):S41–S48, 2002). We present a patient who developed a tremor, mostly involving her lower extremities, secondary to an ischemic infarct affecting the cerebellum, thalamus and midbrain. Attempts at medical treatment with levodopa, quetiapine and levetirecetam all failed. However, pribedil, a dopaminergic receptor-stimulating agent, successfully improved the tremor. Our case is interesting as published reports generally focus on tremors limited to the upper extremities except for one reported case of Holmes' tremor involving the lower extremities more severely (Walker et al. in Mov Disord 22(2):272–274, 2007). It also demonstrates that dopaminergic receptor stimulating agents should be tested before considering invasive therapies.     

Efficacy of sertraline in a patient with Neuro-Beçhet’s disease

Behçet’s disease (BD) is a heterogeneous multisystem inflammatory disorder of unknown etiology, of which the involvement of the central nervous system is a serious manifestation (Neuro-Behçet’s syndrome, NBS). We report a 65-year-old patient who presented with a progressive cognitive impairment and behavioral disturbances (depression and apathy). He fulfilled the International Study Group criteria for BD. Treatment with sertraline was then commenced with an objective improvement of his cognitive and behavioral status. The current report describes a patient with an extensive history of NBS (almost 20 years) and a possible therapeutic option for behavioral impairment.     

Patterns of intrinsic brain activity in essential tremor with resting tremor and tremor-dominant Parkinson’s disease

Brain Imaging and Behavior - The clinical pictures of essential tremor (ET) with resting tremor (rET) and tremor-dominant Parkinson’s disease (tPD) are often quite mimic at the early stage,...     

Treatment of Wernicke’s encephalopathy with high dose of thiamine in a patient with pyloric sub-stenosis: description of a case

Wernicke’s encephalopathy (WE) is an acute or subacute syndrome that results from a deficiency in vitamin B1 (thiamine). The syndrome is characterised by a classical triad of symptoms: nystagmus and ophthalmoplegia, mental-status changes, and unsteadiness of stance and gait. When patients with WE are inappropriately treated with low doses of thiamine, mortality rates average out at 20% and Korsakoff’s Psychosis develops in about 85% of survivors (Sechi and Serra in Lancet Neurol 6(5):442–455, 2007). We report the case of a patient with a pyloric sub-stenosis that developed a WE, and was treated with high doses of thiamine showing after few days of treatment a great improvement of neurological and neuroradiological assessment, even though cognitive impairment was still severe at discharge and at 6 months follow-up.     

Recurrent sigmoid volvulus in a patient with Parkinson’s disease

Clinical Autonomic Research -     

Deep brain stimulation in the treatment of Holmes tremor – A long-term case observation

We present the patient with Holmes tremor secondary to the infarction of thalamus, successfully treated with the deep brain stimulation (DBS) of the area between ventralis oralis anterior and zona incerta for a long time, in whom the severe tremor reappeared after removal of the DBS lead. This is the first presentation of the effective DBS on this location. Our case does not support the hypothesis that the DBS treatment could lead to sustained relief of symptoms after cessation of stimulation.     

Unusual ‘Spike-wave stupor’ in a patient with manic-depressive psychosis treated with amitriptyline

Summary Spike-wave stupor was observed in a 58-year-old male patient with manic-depressive psychosis. Almost continuous atypical spike-wave activity was seen in conjunction with a stuporous episode with stereotyped automatism. Intravenous diazepam ended both the electroencephalographic epileptiform discharges and the clinical stupor. Before and during this episode the patient was treated with an average-dose amitriptyline monotherapy. There was no family history of epileptic seizures. The patient had had electroconvulsive therapy. The history suggests that the analeptic property of amitriptyline induced the spike-wave stupor in this patient.

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Zusammenfassung Während eines Stupors mit stereotypen Bewegungsabläufen und Reihensprechen, der im Rahmen einer depressiven Phase eines an einer manisch-depressiven Psychose erkrankten 58jährigen Patienten auftrat, wurde eine nahezu kontinuierliche unregelmäßige spike-wave- und sharp-wave-Aktivität im EEG abgeleitet. Der spike-wave-Stupor konnte durch eine intravenöse Applikation von Diazepam in seiner klinischen und elektroenzephalographischen Ausprägung rasch unterbrochen werden. Vor und während der stuporösen Attacke wurde der Patient mit Amitriptylin als Monotherapie in üblicher Dosierung behandelt. Die Eigen- und Familienanamnese ergab keinen Hinweis auf das Vorliegen einer Epilepsie. Epileptische Anfälle hatte der Patient nur aus therapeutischen Gründen im Rahmen einer Elektroschocktherapie. Die Analyse des Falles läßt in der analeptischen Wirkung von Amitriptylin die Ursache für das Auftreten des spike-wave-Stupors erkennen.

     

Glial intranuclear inclusion bodies in a patient with Alzheimer’s disease

We report a case of dementia in an elderly woman with the pathological findings of Alzheimer’s disease and numerous intranuclear inclusions in astrocytes and occasionally in neurons. These inclusions were seen in the cerebral cortex, limbic areas, basal ganglia, thalamus, brain stem and cerebellum. They expressed ubiquitin and were ultrastructurally composed of haphazardly arranged straight filaments. The presence of similar intranuclear inclusions in previous cases of adult-onset dementia without other neuropathological changes suggests an important link between these kind of inclusions and dementia. To our knowledge, this type of intranuclear inclusions has not been previously described in association with Alzheimer pathology. Received: 28 July 1999 / Accepted: 15 September 1999     

A longitudinal study of tremor frequencies in Parkinson’s disease and essential tremor

ObjectiveThere is evidence that the tremor frequency in essential tremor (ET) decreases with time. Longitudinal studies on the evolution of tremor frequencies in Parkinson’s disease (PD) have so far not been published. Here, we present a longitudinal analysis of tremor frequencies in PD and ET.MethodsWe analyzed the standardized accelerometric and electromyographic tremor recordings of 53 patients with PD and 38 patients with ET who underwent repeated routine tremor recordings between 1991 and 2002.ResultsIn an average follow-up period of 44.9 months in PD and 50.6 months in ET, the average number of tremor recordings was 3.3 in PD and 3.7 in ET. In both disorders, tremor frequencies tended to decrease with time. The average annual decrease of the tremor frequency was 0.09 Hz/year in Parkinsonian rest tremor, 0.08 Hz/year in Parkinsonian postural tremor and 0.12 Hz/year in ET.ConclusionsThe tremor frequency decreases with time in both PD and ET. The similarity of this decrease in PD and ET may point to a common underlying pathophysiological mechanism.SignificanceDecreasing tremor frequencies with time may be functionally important by inducing larger tremor amplitudes due to the low-pass filtering properties of muscles and limbs.     

Incidence of Parkinson’s disease in a large patient cohort with idiopathic smell and taste loss

Journal of Neurology - Patients with idiopathic smell loss constitute an at-risk population for the development of Parkinson’s disease (PD). The study aimed to follow up a large number of...     

Myoclonus and tremor response to thalamic deep brain stimulation parameters in a patient with inherited myoclonus–dystonia syndrome

We present a 74-year-old woman with inherited myoclonus–dystonia, with predominant myoclonus and a novel mutation in the ?-sarcoglycan gene. The patient reports a life-long history of rapid, jerking movements, most severe in the upper extremities as well as a postural and action tremor. Bilateral deep brain stimulation (DBS) of the ventral intermediate nucleus of the thalamus was performed, and the patient demonstrated moderate clinical improvement in myoclonus. We studied the effects on myoclonus and tremor of varying DBS frequency and amplitude. The frequency tuning curve for myoclonus was similar to that of tremor, suggesting similar mechanisms by which DBS alleviates both disorders.