Monthly Archives: July 2010

This is an interesting German study of dysphagia (swallowing problems) in PSP.

“Dysphagia is…a common feature in PSP occurring in up to 80% of patients. In comparison to Parkinson’s disease (PD), median dysphagia latency and survival time after onset of dysphagia are significantly shorter in PSP. Dysphagia may result in aspiration and subsequent pneumonia, which is the leading cause of death in both disorders. Other complications of swallowing dysfunction are discomfort while eating, difficulties in taking oral medications, weight loss, and dehydratation. Furthermore, swallowing disturbance is associated with increased anxiety and depression and significantly affects the quality of life in parkinsonian syndromes. Therefore, early detection and timely interventions are mandatory to prevent the serious consequences of dysphagia.”

In this study, the FEES test was used: fiberoptic endoscopic evaluation of swallowing. (The R in FEES(R) is “registered trademark.”) The FEES test includes a flexible fiberoptic endoscope in put down the nose, providing a view of the swallowing structures.

Eighteen PSP patients and 15 advanced stage PD (Parkinson’s Disease) patients were given this test. Seven of the PSP patients were tested after they had been given levodopa (Sinemet is a brand name). “Two PSP patients showed relevant improvement of swallowing function after L-dopa challenge.” One patient was able to return to normal food and the other patient had a nasogastric tube removed.

“Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients.”

“In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing. … More than half of these patients did not recognize their disability. These observations may suggest that liquid leakage resulting from a disturbed oral swallowing phase is another early and under diagnosed ancillary clinical attribute in PSP differentiating this condition from PD.”

“Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. … There were no significant differences between the PSP and PD patients.”

This finding was interesting: “No correlation was found with dysarthria severity.” I would’ve thought that dysarthria severity and dysphagia severity were highly correlated.

And this finding was also interesting: “Significant pharyngeal saliva pooling was observed in 4 PSP patients.” It has been shown that “pharyngeal saliva pooling strongly predicts severe dysphagia and substantially increases the risk for the development of aspiration pneumonia.”

“Chin tuck-maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions.” Two examples of food consistency modification are restriction to pureed food or restriction to soft solid food. “[In] certain PSP patients with delayed swallowing reflex [a] hard swallow may not be an appropriate maneuver, because it potentially further increases the duration of pharyngeal response.”

The researchers had something to say about PEG feeding tubes. “Although several severely affected PSP patients participated in our study, only two subjects required percutaneous endoscopic gastrostomy. In our opinion, a percutaneous endoscopic gastrostomy should be considered in PSP patients when there is endoscopic or videofluoroscopic evidence that all food consistencies are aspirated without any objective improvement by L-dopa challenge and/or nonpharmacological therapeutic interventions.”

Robin

Movement Disorders. 2010 Mar 29. [Epub ahead of print]

Endoscopic characteristics and levodopa responsiveness of swallowing function in progressive supranuclear palsy.

Warnecke T, Oelenberg S, Teismann I, Hamacher C, Lohmann H, Ringelstein EB, Dziewas R.
Department of Neurology, University Hospital of Münster, Münster, Germany.

Abstract
Dysphagia is a frequent and early symptom in progressive supranuclear palsy (PSP) predisposing patients to aspiration pneumonia. Fiberoptic endoscopic evaluation of swallowing (FEES(R)) has emerged as a valuable apparative tool for objective evaluation of neurogenic dysphagia. This is the first study using FEES(R) to investigate the nature of swallowing impairment in PSP.

Eighteen consecutive PSP patients (mean age 69.7 +/- 9.0 years) were included. The salient findings of FEES(R) in PSP patients were compared with those of 15 patients with Parkinson’s disease (PD). In 7 PSP patients, a standardized FEES(R) protocol was performed to explore levodopa (L-dopa) responsiveness of dysphagia.

Most frequent abnormalities detected by FEES(R) were bolus leakage, delayed swallowing reflex, and residues in valleculae and piriformes. Aspiration events with at least one food consistency occurred in nearly 30% of PSP patients. Significant pharyngeal saliva pooling was observed in 4 PSP patients.

We found no difference of salient endoscopic findings between PSP and PD patients.

Endoscopic dysphagia severity in PSP correlated positively with disease duration, clinical disability, and cognitive impairment. No correlation was found with dysarthria severity. In early PSP patients, swallowing dysfunction was solely characterized by liquid leakage with the risk of predeglutitive aspiration during the oral phase of swallowing.

Two PSP patients showed relevant improvement of swallowing function after L-dopa challenge.

Chin tuck-maneuver, hard swallow, and modification of food consistency were identified as the most effective therapeutic interventions.

In conclusion, FEES(R) assessment can deliver important findings for the diagnosis and refined therapy of dysphagia in PSP patients.

(c) 2010 Movement Disorder Society.

PubMed ID#: 20629130 (see pubmed.gov for this abstract only)

Dr. Richard Armstrong has written extensively on visual impairment in PSP and other parkinsonism syndromes.  His latest article for optometrists is a good general review of PSP, followed by a review of the visual signs that can be used to identify PSP, and a comparison between these PSP visual symptoms and those of other movement disorders, especially Parkinson’s Disease (PD).

Dr. Armstrong states:

Although primarily a neurological disorder, patients with PSP may develop a variety of visual signs and symptoms that could be useful in differential diagnosis. In addition, a PSP patient may develop visual problems during the course of the disease and the eye-care practitioner can contribute to the management of these problems.

He examines these many “ocular aspects,” including eyelid mobility (such as apraxia of lid opening, blepharospasm), fixation, ophthalmoplegia (such as vertical supranuclear gaze palsy), saccades, smooth pursuit movements, nystagmus (including square wave jerks), and reading.

Here is Dr. Armstrong’s list of practical ways an optometrist can help a PSP patient:

• “PSP patients tend to fall more readily, resulting in a sedentary life style, while vertical gaze palsy also leads to difficulties with reading and eating. New devices are being tested in which refraction positive moveable prism glasses are placed in front of the eyes to help patients with these problems.”
• “PSP patients often complain of dry eyes due to decreased blinking and artificial tears can be used to help this problem.”
• “Patients with difficulties in opening their eyelids may have ‘lid crutches’ fitted to spectacle frames that can hold the lids open.”
• “[Blepharospasm] has been treated successfully with botulinum toxin A…”
• “PSP patients have a particular difficulty with vertical saccades and in suppressing fixation, both of which may contribute to vertical gaze palsy. Deficits in gaze control may … increase the risk of trips or falls while climbing stairs. Balance training in combination with eye movement and visual awareness exercises have been shown to improve gaze control in PSP patients. Patients can be referred to physiotherapists or occupational therapists.”
• “Some patients with PSP may have been treated with L-dopa… Some patients treated with L-dopa exhibit a worsening of apraxia of eye opening as a result, a condition that is often alleviated when the drug is discontinued. By contrast, treatment with apomorphine may reduce apraxia of eye opening.”
• “[Visual] problems that cannot be corrected, such as oculomotor apraxia or visual field defects, should be brought to the attention of carers. As a consequence of such information, carers can make accommodations for these problems, for example, by careful positioning of objects.”

I’ve copied the abstract below along with a link to the full article, which is available at no charge online.

Robin

Full article

Abstract

Clinical and Experimental Optometry. 2010 Jul 12.
Visual signs and symptoms of progressive supranuclear palsy.
Armstrong RA.
Vision Sciences, Aston University, Birmingham, UK

Progressive supranuclear palsy is a rare, degenerative brain disorder and the second most common syndrome in which the patient exhibits ‘parkinsonism’, that is, a variety of symptoms involving problems with movement. General symptoms include difficulties with gait and balance; the patient walking clumsily and often falling backwards. The syndrome can be difficult to diagnose and visual signs and symptoms can help to separate it from closely related movement disorders such as Parkinson’s disease, multiple system atrophy, dementia with Lewy bodies and corticobasal degeneration. A combination of the presence of vertical supranuclear gaze palsy, fixation instability, lid retraction, blepharospasm and apraxia of eyelid opening and closing may be useful visual signs in the identification of progressive supranuclear palsy. As primary eye-care practitioners, optometrists should be able to identify the visual problems of patients with this disorder and be expected to work with patients and their carers to manage their visual welfare.

PubMed ID#: 20629667

This is an important review article from our friends at the Mayo Clinic.  Researchers examine the differences in distribution of tau pathology and differences in biochemical composition of tau pathology in at least two of the variants, as compared to “classic” or typical progressive supranuclear palsy (PSP).

They find that:

• “In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP.”
• “In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei – globus pallidus, subthalamic nucleus and substantia nigra – than in typical PSP.”

The full abstract is copied below.

Robin

——————————

Current Opinion in Neurology. 2010 Aug;23(4):394-400.

Neuropathology of variants of progressive supranuclear palsy.

Dickson DW, Ahmed Z, Algom AA, Tsuboi Y, Josephs KA.

Abstract
PURPOSE OF REVIEW:
Neurodegenerative tauopathies, of which progressive supranuclear palsy (PSP) is one of the most common, are clinically heterogeneous, reflecting differences in distribution and biochemical composition of tau pathology. This review highlights the range of clinical and pathologic presentations of PSP and its variants.

RECENT FINDINGS:
Progressive supranuclear palsy is a 4R tauopathy with neuronal and glial tau-immunoreactive lesions in neuroanatomically specific nuclei in the basal ganglia, diencephalon, brainstem and cerebellum, with restricted involvement of the neocortex. Hierarchical cluster analyses of clinical and pathologic features of PSP indicate that there are distinct clinicopathologic variants of PSP. In variants of PSP presenting with focal cortical syndromes, such as frontotemporal dementia, corticobasal syndrome and apraxia of speech, there is greater cortical pathology than in typical PSP. In variants of PSP presenting with levodopa-responsive Parkinsonism, as well as pure akinesia and gait failure, there is less cortical pathology and more severe degeneration in the cardinal nuclei – globus pallidus, subthalamic nucleus and substantia nigra – than in typical PSP.

SUMMARY:
Clinical variants in PSP reflect varying anatomical distribution of tau pathology, but they share histopathologic, biochemical and genetic features with typical PSP. The basis for anatomical selective vulnerability in PSP and its variants remains to be determined.

PubMed ID#: 20610990 (see pubmed.gov for this abstract – available free of charge)