Frequency and outcomes of gastrostomy insertion in a longitudinal cohort study of atypical parkinsonism.

BACKGROUND: Multiple system atrophy (MSA), progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) show a high prevalence and rapid progression of dysphagia, which is associated with reduced survival. Despite this, the evidence base for gastrostomy is poor, and the optimal frequency and outcomes of this intervention are not known. We aimed to characterise the prevalence and outcomes of gastrostomy in patients with these three atypical parkinsonian disorders. METHOD: We analysed data from the natural history and longitudinal cohorts of the PROSPECT-M-UK study with up to 60 months of follow-up from baseline. Survival post-gastrostomy was analysed using Kaplan-Meier survival curves. RESULTS: In a total of 339 patients (mean age at symptom onset 63.3 years, mean symptom duration at baseline 4.6 years), dysphagia was present in >50% across all disease groups at baseline and showed rapid progression during follow-up. Gastrostomy was recorded as recommended in 44 (13%) and performed in 21 (6.2%; MSA 7, PSP 11, CBS 3) of the total study population. Median survival post-gastrostomy was 24 months compared with 12 months where gastrostomy was recommended but not done (p = 0.008). However, this was not significant when correcting for age and duration of symptoms at the time of procedure or recommendation. CONCLUSIONS: Gastrostomy was performed relatively infrequently in this cohort despite the high prevalence of dysphagia. Survival post-gastrostomy was longer than previously reported, but further data on other outcomes and clinician and patient perspectives would help to guide use of this intervention in MSA, PSP and CBS.

[Clinical features and effects of shunt surgery in patients with progressive supranuclear palsy and idiopathic normal pressure hydrocephalus].

This study aimed to retrospectively review the frequency and clinical features of 13 patients with progressive supranuclear palsy (PSP) and idiopathic normal pressure hydrocephalus (iNPH). All patients were found to have PSP-Richardson's syndrome (PSP-RS). Shunt surgery was effective in 5 of 11 patients (45.5%). A comparison of these 5 patients who responded to shunt surgery versus the remaining 6 patients revealed a significant difference in the reduction of frontal lobe blood flow on cerebral perfusion single-photon emission computed tomography (SPECT) (P = 0.018). These results suggest that PSP-RS is common in patients with PSP and iNPH and indicate the usefulness of cerebral perfusion SPECT in estimating the effect of shunt surgery.

Neuronal spiking in the pedunculopontine nucleus in progressive supranuclear palsy and in idiopathic Parkinson's disease.

The pedunculopontine nucleus (PPN) is engaged in posture and gait control, and neuronal degeneration in the PPN has been associated with Parkinsonian disorders. Clinical outcomes of deep brain stimulation of the PPN in idiopathic Parkinson's disease (IPD) and progressive supranuclear palsy (PSP) differ, and we investigated whether the PPN is differentially affected in these conditions. We had the rare opportunity to record continuous electrophysiological data intraoperatively in 30 s blocks from single microelectrode contacts implanted in the PPN in six PSP patients and three IPD patients during rest, passive movement, and active movement. Neuronal spikes were sorted according to shape using a wavelet-based clustering approach to enable comparisons between individual neuronal firing rates in the two disease states. The action potential widths showed a bimodal distribution consistent with previous findings, suggesting spikes from noncholinergic (likely glutamatergic) and cholinergic neurons. A higher PPN spiking rate of narrow action potentials was observed in the PSP than in the IPD patients when pooled across all three conditions (Wilcoxon rank sum test: p = 0.0141). No correlation was found between firing rate and disease severity or duration. The firing rates were higher during passive movement than rest and active movement in both groups, but the differences between conditions were not significant. PSP and IPD are believed to represent distinct disease processes, and our findings that the neuronal firing rates differ according to disease state support the proposal that pathological processes directly involving the PPN may be more pronounced in PSP than IPD.

Surgical treatment of vertical ocular motility disorders of supranuclear origin.

Vertical ocular motility disorders of supranuclear origin can be congenital or acquired later in life. They produce complex forms of strabismus that require specialized surgical management to restore ocular alignment, eliminate torticollis, treat diplopia, and restore normal lid position. For each condition, surgical management must be individualized to address multiple impediments to fusion. This article outlines the surgical management of three common types of supranuclear vertical strabismus.

Anesthetic management of a patient with progressive supranuclear palsy.

A 74-year-old woman with progressive supranuclear palsy (PSP) was scheduled for laryngotracheal separation surgery. Her neck showed severe backward tilt as a symptom of PSP. Magnetic resonance imaging (MRI) showed a significant airway stenosis due to the neck deformity. In the operating room, awake orotracheal intubation failed because of the neck deformity and airway stenosis. Therefore, tracheotomy was performed for airway management. General anesthesia was induced and maintained with sevoflurane (1.0%-2.5%) and fentanyl (total, 200 microg). Vecuronium (total, 5 mg) was used as a muscle relaxant. Monitoring of the train-of--four ratio in the ulnar nerve was impossible because of contracture of the fingers. Patients with PSP may have some serious associated deformities, and specific management, especially for the airway, may be necessary for general anesthesia.

The internal globus pallidus is affected in progressive supranuclear palsy and Parkinson's disease.

Our structural studies of the substantia nigra in parkinsonian patients identified previously unsuspected changes in the pars reticulata, suggesting significant dysfunction in this basal ganglia output. There have been few similar structural studies of the other major basal ganglia output, the internal segment of the globus pallidus. This is despite significant evidence that this basal ganglia region is crucially important for generating parkinsonian symptoms. In fact current surgical interventions target this region in Parkinson's disease. The cellular anatomy of the internal globus pallidus was compared among five controls, six patients with Parkinson's disease, and five patients with progressive supranuclear palsy. Neurons and pathological structures were quantified using the unbiased fractionator method. Only cases with progressive supranuclear palsy had detectable pathology within the internal globus pallidus in the form of tau-positive neuronal and glial tangles and substantial neurodegeneration. Cases with Parkinson's disease had a significant reduction in the proportion of neurons containing parvalbumin but were without significant neurodegeneration, consistent with dysfunction of both basal ganglia output nuclei in advanced parkinsonism. Surgical ablation of the internal globus pallidus for Parkinson's disease appears at odds with the significant neurodegeneration in the similarly akinetic and rigid patients with progressive supranuclear palsy. The results are discussed in association with current hypotheses of basal ganglia function and recent experimentation in patients undergoing pallidotomy for hyperkinetic disorders.

High incidence of cardiopulmonary complications associated with implantation of adrenal medullary tissue into the caudate nucleus in patients with advanced neurologic disease.

OBJECTIVE: The purpose of our study was to examine the cardiopulmonary complications of a group of patients who had undergone implantation of adrenal medullary tissue into the caudate nucleus for treatment of neurologic disease. DESIGN: Prospective study with partially matched historical controls. SETTING: Tertiary care community medical center. PATIENTS AND METHODS: Seven patients with advanced Parkinson's disease and three patients with progressive supranuclear palsy underwent implantation of adrenal medullary tissue into the caudate nucleus. These patients were compared with respect to their cardiopulmonary complications with a control group who had undergone craniotomy and then compared with a control group who had undergone only abdominal surgery. RESULTS: In the study group, six patients developed major postoperative complications including development of large pleural effusions, lobar atelectasis, pneumonia, upper airway obstruction, and cardiac arrest. Three patients had minor complications including development of small pleural effusions, subsegmental atelectasis, purulent bronchitis, mild congestive heart failure, and atrial flutter/fibrillation. One patient had an unremarkable postoperative course. The first control group, whose only surgery was a craniotomy, had only one major complication. The second control group, the abdominal surgery control group, had one major and five minor complications. CONCLUSION: The particular neurologic disease, its severity, and the type of surgery performed appear to be causative factors in the high incidence of complications in the study group.

Autologous transplantation as a treatment for progressive supranuclear palsy.

Autologous transplantation of the adrenal gland has been attempted as a treatment for severe Parkinson's disease with limited success. Progressive supranuclear palsy (PSP), a more aggressive neurological entity of similar pathology, is due to a loss of dopamine in the caudate nucleus and putamen. The autologous transplant procedure has been performed on three PSP patients in an effort to restore dopamine to the caudate nucleus and putamen. The rationale and preliminary results of the transplantation procedure are reviewed in this article.

Autologous adrenal medullary transplant in progressive supranuclear palsy.

We transplanted autologous adrenal medullary cells to the caudate nucleus in 3 patients with progressive supranuclear palsy, using the method Madrazo has employed for neural transplantation in Parkinson's disease. Major and minor complications occurred post-operatively from which the patients recovered. One patient had a marked improvement in his postural stability and a decreased incidence of falling. This change was evident at 1 month after surgery and has remained for the 6 months of follow-up. Postural reflexes were not altered in the other 2 patients. There was no change in extraocular movements, speech, or the rigid-bradykinetic features of parkinsonism in any patient. Adrenal medullary transplantation has only limited efficacy in progressive supranuclear palsy.