Re:CMT 1A patients with low level of impairment have a higher energy cost

[Guest guest]

This was a very interesting article. I remember reading another

article recently with similar information. To a large extent this

describes me. I have " no clinically evident walking impairment " .

But I do walk noticeably slower than other people when at the mall,

out for a walk, etc. A few weeks ago we went to the airport to pick

up a friend. He and my husband took off walking through the airport

to get to the parking lot. I felt like I almost had to run to keep

up with them. I don't get winded, but just can't seem to take long

enough strides to keep pace. It's kind of funny, because about 20

years ago I was involved in some walking races (informal events),

where I regulary won. But there's no way I could do that now.

However, at this point, I don't trip at all, and my ankles never give

out when walking so I am thankful for that. Thanks for sending the

abstract of this article. CMT1 is the best guess I have for my

subtype, based on my EMG results.

Also, I will be returning to the USA next month for a few months, and

am in the process of setting up an appointment with a neurologist who

specializes in neuropathies. I will also be seeing an internist

friend who has experience in both CMT and CIDP. I want to review the

EMG and lumbar puncture results, and see what they have to say. I

find it interesting that the one neurologist here said he thinks

there may be some immune issues involved, not just CMT. I know that

sometimes my left leg feels a bit numb all the way up to the top of

my leg, and don't think CMT affects the leg that far up. The

numbness comes and goes- usually feel it more in the morning after I

get up- so maybe it has something to do with the way I am sleeping.

Blessings,

Barb

South Africa

Neuromuscul Disord. 2010 Nov 11

Charcot-Marie-Tooth 1A patients with low level of impairment have a

higher energy cost of walking than healthy individuals.

Menotti F, Felici F, Damiani A, Mangiola F, Vannicelli R, Macaluso A.

Department of Human Movement and Sport Sciences, University of Rome

Foro Italico, Rome, Italy.

Abstract

The study aimed at quantifying the walking energy cost of a group of

Charcot-Marie-Tooth 1A patients (CMT1A), with low severity of walking

impairment, in comparison with healthy individuals. Oxygen uptake was

measured in 8 patients (age-range 20-48years; Barthel >90; Tinetti

>20) and 8 healthy individuals, matched for age and gender, when

walking on a circuit for 5-min at their self-selected speeds ( " slow " ,

" comfortable " and " fast " ). Both comfortable and fast speeds were

lower in patients than in the control group (0.92±0.16 vs 1.16±0.22

and 1.27±0.27 vs 1.61±0.22ms(-1), respectively; P<0.05), whereas

walking energy cost per unit of distance was higher in patients than

in the control group (P<0.05) at both " comfortable " (2.27±0.35 vs

1.92±0.21Jkg(-1)m(-1)) and " fast " speed (3.05±0.35 vs 2.37±0.42Jkg(-

1)m(-1)).

CMT1A patients, therefore, choose to walk slower but with higher

metabolic cost compared to healthy individuals, despite no clinically

evident walking impairment, which is likely due to altered walking

patterns.