“Mind, Mood & Memory” booklet

This terrific booklet, Parkinson’s Disease: Mind, Mood & Memory, was published several years ago by the National Parkinson Foundation (parkinson.org).  I hand it out to all newcomers to our Lewy Body Dementia caregiver-only support group meetings.  It has a wonderful chapter (five) focused on Dementia with Lewy Bodies.

The booklet makes the important point that there’s a delicate balance between treating psychosis (hallucinations and delusions) and treating parkinsonian motor symptoms.

Here’s a link to it:

www3.parkinson.org/site/DocServer/Mind_Mood_Memory.pdf?docID=191

I hope you value this booklet as much as I do!

Robin

Now 2 Autonomic Dysfunction Specialists in Northern CA

Finally, there’s a neurologist who is an autonomic specialist in the Bay Area.  His name is Safwan Jaradeh, MD.  He’s at Stanford.  (Neurology appointment phone is 650-723-6469.  New patient appointment phone is 650-725-5792.  New patients will need a referral from a neurologist.)  He used to be at the Medical College of Wisconsin.  A patient’s family who saw him in WI wrote a very positive review of him on POTSplace (www.potsplace.com/physicians.htm).

The only other MD in the Bay Area who sees patients with autonomic dysfunction is a cardiologist named Karen Friday, MD.  She’s at Stanford (Cardiology) and the Palo Alto VA.  A couple of years ago one of our MSA group members was receiving inadequate treatment for OH (orthostatic hypotension) from a movement disorder specialist.  The group member took her husband to see Dr. Friday and was very impressed with the care received.  Also, someone wrote a positive review of her on POTSplace.

Years ago, someone told me:  “Many of the autonomic specialists are cardiologists rather than neurologists, because of the close association between the autonomic system and blood pressure.”

If any of you are dealing with or have dealt with SEVERE symptoms of orthostatic hypotension and you are receiving excellent care for these challenging symptoms from your neurologist, please let me know.  Thus far, I’ve only heard negative stories about neurologists and OH treatment.

Robin

PS. There are three directories of autonomic specialists in the US:

www.potsplace.com/physicians.htm
www.ndrf.org/physicia.htm
www.mc.vanderbilt.edu/gcrc/aas/AAS_referals_by_state.htm#CA

2 main types of PSP have different FDG-PET scan findings

There is a special type of PET scan — fluorodeoxyglucose PET (FDG-PET) — that tests for glucose metabolism in the brain.  Such scans are only available at large medical research institutions, such as UCSF or Stanford.  There have been FDG-PET studies in the past comparing progressive supranuclear palsy (PSP) and Parkinson’s Disease patients.

This German study takes the research a step further and looks at two different types of PSP.  In this study, 11 patients with the Richardson’s syndrome (RS) form of PSP, 8 patients with the parkinsonism form of PSP (PSP-P), 12 patients with Parkinson’s Disease, and 10 controls underwent an FDG-PET.  They found that different areas in the brain were affected in each of the PSP patient groups — thalamus and frontal lobe for RS and putamen for PSP-P.

For those who may be new to our PSP support group, let me briefly give the primary symptoms of these two most common types of PSP:

* RS, or “classic PSP”:  early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction

* PSP-P:  asymmetric onset, tremor, a moderate initial therapeutic response to levodopa, and frequently confused with Parkinson’s disease

If you’d like to read more about the two most common types of PSP, see this post from 2005:

www.brainsupportnetwork.org/two-distinct-types-of-psp-rs-and-psp-parkinsonism/

Using this German study, we can correlate some of these primary symptoms of the two types of PSP with the FDG-PET findings:

* Reduced neurotransmitter activity levels in the thalamus are associated with falls and postural instability, and those with RS have a high frequency of falls.

* Reduced activity in the frontal lobe is associated with cognitive dysfunction, which occurs in those with RS.  (RS patients “have an increased risk for dementia.”)

* And the putamen is associated with parkinsonism symptoms, such as those seen in PSP-P.  Frontal metabolism was normal for PSP-P patients.

The authors state that the “putamen/thalamus ratio may be a useful parameter in clinical differential diagnosis of these PSP subgroups.”  This is potentially helpful for distinguishing PSP-P from PD (Parkinson’s Disease) because these two diseases are hard to differentiate in the early stages.

Limitations of this study are the small sample size and the lack of pathological confirmation of the diagnoses.  I’ve copied the abstract below.

Robin

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Movement Disorders. 2012 Jan;27(1):151-5.

Fluorodeoxyglucose positron emission tomography in Richardson’s syndrome and progressive supranuclear palsy-parkinsonism.

Srulijes K, Reimold M, Liscic RM, Bauer S, Dietzel E, Liepelt-Scarfone I, Berg D, Maetzler W.
Department of Neurodegeneration, Hertie Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany.

Abstract
BACKGROUND:
We hypothesized that postural instability and cognitive decline in patients with Richardson’s syndrome could be a consequence of reduced thalamic and frontal metabolism. Severe Parkinsonian signs in patients with progressive supranuclear palsy-parkinsonism may be reflected by alterations in putaminal metabolism.

METHODS:
Eleven patients with Richardson’s syndrome, 8 patients with progressive supranuclear palsy-parkinsonism, 12 with Parkinson’s disease, and 10 controls underwent clinical assessment and fluorodeoxyglucose positron emission tomography (PET).

RESULTS:
Richardson’s syndrome patients showed pronounced thalamic hypometabolism, and patients with progressive supranuclear palsy-parkinsonism pronounced putaminal hypometabolism, compared to all other investigated groups. The putamen/thalamus uptake ratio differentiated progressive supranuclear palsy-parkinsonism from Richardson’s syndrome (area under the curve 5 0.86) and from Parkinson’s disease (area under the curve 5 0.80) with acceptable accuracy. Frontal hypometabolism was predominantly found in Richardson’s syndrome patients.

CONCLUSIONS:
Richardson’s syndrome, progressive supranuclear palsy-parkinsonism and Parkinson’s disease showed different metabolic patterns in fluorodeoxyglucose PET.

PubMed ID#:  #22359740  (see https://www.ncbi.nlm.nih.gov/pubmed/?term=22359740 for this abstract only)

Dopamine Transporter Loss Differs in MSA, PSP and PD

With a special PET scan that focuses on dopamine transport, these Korean researchers concluded that PSP (progressive supranuclear palsy) patients showed “more prominent and earlier dopamine transporter loss” in the anterior caudate, a region of the brain, and that MSA (multiple system atrophy) patients showed “more prominent and earlier dopamine transporter loss” in the ventral putamen, another region of the brain, when compared to Parkinson’s Disease.

None of the diagnoses are autopsy-confirmed, which is what is needed for the medical community to embrace this sort of PET scan.

I’ve copied the abstract below.

Robin

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Journal of Nuclear Medicine. 2012 Feb 9. [Epub ahead of print]

Subregional Patterns of Preferential Striatal Dopamine Transporter Loss Differ in Parkinson Disease, Progressive Supranuclear Palsy, and Multiple-System Atrophy.

Oh M, Kim JS, Kim JY, Shin KH, Park SH, Kim HO, Moon DH, Oh SJ, Chung SJ, Lee CS.
Department of Nuclear Medicine, Asan Medical Center, College of Medicine, University of Ulsan, Seoul, Korea.

Abstract
Parkinson disease (PD), progressive supranuclear palsy (PSP), and multiple-system atrophy (MSA) are known to affect dopaminergic neurons of the brain stem and striatum with different preferential involvement. Here we investigated differences in striatal subregional dopamine transporter loss in PD, PSP, and MSA and assessed the diagnostic value of (18)F-fluorinated-N-3-fluoropropyl-2-beta-carboxymethoxy-3-beta-(4-iodophenyl)nortropane ((18)F-FP-CIT) PET in differentiating PSP and MSA from PD.

METHODS:
Forty-nine patients with PD, 19 patients with PSP, 24 patients with MSA, and 21 healthy people (healthy controls) were examined with (18)F-FP-CIT PET.

The PET images were spatially normalized and analyzed with 12 striatal subregional volume-of-interest (VOI) templates (bilateral ventral striatum [VS], anterior caudate [AC], posterior caudate, anterior putamen, posterior putamen [PP], and ventral putamen [VP]) and 1 occipital VOI template. The nondisplaceable binding potential (BP(ND)) and intersubregional ratio (ISR; defined as the ratio of the BP(ND) of one striatal subregion to that of another striatal subregion) of subregional VOIs were calculated.

RESULTS:
The BP(ND) of all VOIs in the PD, MSA, and PSP groups were significantly lower than those in the healthy controls (P < 0.05). The BP(ND) of AC and the AC/VS ISR in the PSP group were significantly lower than those in the PD group. The BP(ND) of VP was significantly lower, but the PP/VP ISR was significantly higher in the MSA group than in the PD group. At the cutoff value for the AC/VS ISR (<0.7), the sensitivity and specificity for differentiating PSP from PD were 94% and 92%, respectively. At the cutoff value for the PP/VP ISR (>0.65), the sensitivity and specificity for differentiating MSA from PD were 90% and 45%, respectively.

The diagnostic accuracy of visual analysis was similar to that of quantitative analysis for differentiating PSP from PD but was significantly higher for differentiating MSA from PD.

CONCLUSION:
Compared with PD, PSP and MSA showed more prominent and earlier dopamine transporter loss in the AC and VP, respectively. These findings could be useful for suggesting PSP or MSA in parkinsonian cases without characteristic atypical features.

PubMed ID#: 22323779 (see pubmed.gov for this abstract only)

Four Markers of Future Neurological Problems

This Italian paper notes that many people develop RBD (REM sleep behavior disorder) and, on average, 25 years later many of them develop a neurological disorder, such as Lewy body dementia (LBD), Parkinson’s Disease (PD), or multiple system atrophy (MSA).  Authors say:

“The estimated 10-year risk of neurodegenerative disease for…RBD is about 40%.” 

Obviously, it would be good to identify who is in that 40% bucket.  The authors indicate that potential markers of neurodegeneration include:

(1) marked EEG slowing on spectral analysis;
(2) decreased striatal 123I-FP-CIT binding and substantia nigra hyperechogenicity;
(3) impaired olfactory function;
(4) impaired color vision.

I was in a meeting once with a neurologist and a local support group member.  The neurologist was reviewing the late husband’s neuropathology report.  I remember the neurologist being very interested in finding the EEGs done on the husband early on as he said that a certain finding on an EEG would’ve been an indicator the husband was going to develop a neurological disorder.”

This is the first time I’ve seen “impaired color vision” as a potential marker.

I’ve copied the abstract of the medical journal article below.

Robin

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Sleep Medicine. 2011 Dec;12 Suppl 2:S43-9.

Does idiopathic REM sleep behavior disorder (iRBD) really exist? What are the potential markers of neurodegeneration in iRBD?

Ferini-Strambi L.
Sleep Disorders Center, Vita-Salute San Raffaele University, Milan, Italy.

Abstract
REM sleep behavior disorder (RBD) may be idiopathic or associated with other neurologic disorders. A strong association between RBD and a-synucleinopathies has recently been observed, with the parasomnia often heralding the clinical onset of the neurodegenerative disease. The idiopathic form accounts for up to 60% of the cases reported in the three largest series of patients with RBD. Some clinical follow-up studies revealed that a large proportion of these patients will eventually develop a parkinsonian syndrome or a dementia of the Lewy bodies type in the years following the RBD diagnosis. The estimated 10-year risk of neurodegenerative disease for idiopathic RBD is about 40%. Moreover, it has been reported that the median interval between RBD and subsequent neurologic syndrome is 25years. Several studies have looked at neurophysiologic and neuropsychological functions in idiopathic RBD and have found evidence of CNS dysfunction during both wakefulness and sleep in a variable proportion of these patients, challenging the concept of idiopathic RBD. Identifying subjects with a high risk of developing a neurodegenerative process may be crucial to develop early intervention strategies. Prospective studies in idiopathic RBD showed that potential markers of neurodegeneration include: (1) marked EEG slowing on spectral analysis; (2) decreased striatal 123I-FP-CIT binding and substantia nigra hyperechogenicity; (3) impaired olfactory function; (4) impaired color vision.

PubMed ID#: 22136899  (see pubmed.gov for this abstract only)