Help with an interesting neuro case

January 24, 2012

I'm hoping that the neuro types (respectfully) and perhaps the PTs who have

dealt with the human counterpart of this disease can help me with this case.

Kaya is a 7 year old Tibetan terrier showing clinical signs of Neuronal Ceroid

Lipofuscinosis – a confirmed Dx with DNA testing. She presently has PRA with

vision loss, is exhibiting fearfulness, and is beginning to stumble. Knuckling

(thanks for making me differentiate from " proprioception " , Steve & #61514;)

provides a slightly slower than N correction but there is no hindlimb or

forelimb dragging, obvious ataxia or other neuro deficits. Obviously the owner

is devastated and I'm wondering how to help her and her dog deal with this

disease for as long as is possible. Questions:

• How much exercise? Tx like DM with no exertion but try to maintain core

strength, ROM and necessary neural pathways?

• Would a Thundershirt or similar help with the fearfulness?

• UWTM? Would the high water temp be a concern?

• Any supplements/herbs that might be beneficial in any way?

• Any other helpful comments either on the disease progression, or how to manage

her until the inevitable?

Thanks in advance to everyone.

Glynis Newman

DVM, CertAVCA, CCRT

January 25, 2012

Glynis there is really very little information on the maintenance of these cases.Here is a little background from human medicine.No specific treatment is available for these diseases.Bone marrow transplant has been tried in animal models as well as a few infants with disappointing results.A study regarding the safety and preliminary efficacy of central nervous system stem cell transplantation in patients with PPT1 or TTP1 deficiency is currently ongoing.Vitamin E, other antioxidants, and selenium have been tried without significant efficacy.Seizures should be treated with standard anticonvulsants. Replication deficient adeno-associated virus gene transfer vector (AAV2-mediated CLN2 gene transfer) has been studied in mice, rats, and nonhuman primates with CLN2. Studying this in children is of interest.Stem cell therapy is actually in some clinical trials http://www.ivis.org/proceedings/acvp/2009/Meisner.pdf?LA=1you would use stem cells from another dog xenograph and use immunosuppressive Tacolimus for immune suppression http://www.jstage.jst.go.jp/article/jvms/68/1/95/_pdfI thought of this as you have a terminal disease not a lot of options and everyone seems to be harvesting mesenchymal stem cells in vet med to treat everything, so they are available.In people with Batten disease an infantile form of lipofuscinosis even a 2% of normal production of palmitoyl protein thioesterase 1 (PPT1) the missing enzyme markedly prolongs life. i think you might have to give the stem cells intrathecally, although I'm obviously not sure. Intravenous might be a first attempt. Just a thought.Anyway:I think heat and exercise would not cause significant issues. I think that the patterning component of UWTM might increase the chance of making new connections in the spinal cord, strengthen the muscles who's pathways are undamaged. I would lean towards lots of exercise as we think might work in DM cases. I would try clorazepate for the fearfulness. It's a benzodiazapine lasts for 8 hours and will also work reasonably well if you have to treat seizures. Like epam it's effectiveness is very individual. It comes in 3.75, & 7.5, & 15 mg tablets. The 7.5's are the easiest to find I'd start at 7.5 mg PO TID and go up or down accordingly. Sedation is virtually the only side effect and although the literature states there can be liver damage I have never seen it and used it hundreds of times. Tough Case, Good Luck,SteveTo: VetRehab From: rheadawn@...Date: Tue, 24 Jan 2012 21:48:17 +0000Subject: Help with an interesting neuro case

I'm hoping that the neuro types (respectfully) and perhaps the PTs who have dealt with the human counterpart of this disease can help me with this case. Kaya is a 7 year old Tibetan terrier showing clinical signs of Neuronal Ceroid Lipofuscinosis – a confirmed Dx with DNA testing. She presently has PRA with vision loss, is exhibiting fearfulness, and is beginning to stumble. Knuckling (thanks for making me differentiate from "proprioception", Steve & #61514;) provides a slightly slower than N correction but there is no hindlimb or forelimb dragging, obvious ataxia or other neuro deficits. Obviously the owner is devastated and I'm wondering how to help her and her dog deal with this disease for as long as is possible. Questions:

• How much exercise? Tx like DM with no exertion but try to maintain core strength, ROM and necessary neural pathways?

• Would a Thundershirt or similar help with the fearfulness?

• UWTM? Would the high water temp be a concern?

• Any supplements/herbs that might be beneficial in any way?

• Any other helpful comments either on the disease progression, or how to manage her until the inevitable?

Thanks in advance to everyone.

Glynis Newman

DVM, CertAVCA, CCRT

January 25, 2012

Hi!

I had a similar case - (determined on necropsy). Unfortunately, we did't get an

antemortem diagnosis because the onset of signs were not consistent with NCL -

and the MRI showed what looked like a mass. On biopsy of the mass - it was

found to be an organized hematoma (which is apparently common with this

disease). We went on to treat symptomatically and did use the T-touch

wrap/thunder shirt - the owners felt that it helped significantly. We also used

alprazolam - and they thought it helped at times when she was inconsolable - and

in the end, helped to sleep at night. I think massage/acupuncture/acupressure

would help - but unfortunately, this is not a good disease and she will likely

continue to deteriorate.

-stephanie

Kube, DVM, DACVIM (Neurology)

Certified Veterinary Pain Practitioner

VCA South Shore Animal Hospital

Weymouth, MA

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> I'm hoping that the neuro types (respectfully) and perhaps the PTs who have