Hello cn-ga,
Your query is about a condition that is not very well understood, and
not even very well defined.
I noted while searching for this information that there seems to be a
lack of consensus about the exact definition of neck extensor muscle
myopathy. Some authors use it as a synonym for dropped head syndrome,
regardless of cause, provided that the relevant muscles are affected.
Others reserve the term for a condition that is non-inflammatory and
mostly non-progressive, ie will not get worse. I have cited papers
which take a broader view, to cover all possibilities. However, I have
not included papers about dropped head syndrome associated with
specific conditions such as Parkinson disease or myasthenia gravis,
because there the treatment will focus on the underlying disease.
Neck extensor muscle myopathy is a condition which weakens the muscles
which hold the head upright. Therefore, the head gradually begins to
sag forward.
A Japanese paper published in 2000 reports one such case. The authors
found that the neck symptoms improved after a month of strict bed
rest. However, when the patient went back to her normal lifestyle,
the symptoms reappeared. The authors concluded “Although the cause of
INEM remains unclear, the present case indicates that the condition is
reversible at least in the early stage of the disease, and the
overloading to the neck extensor muscles is an aggravating factor of
the neck drop in INEM.” Rinsho Shinkeigaku 2000 September, vol 40 (no
9) p.933-936
“Overloading to neck extensor muscles is an aggravating factor to
induce further neck drop in isolated neck extensor myopathy (Katz). A
case report”
[Article in Japanese]
Oishi K, Shigeto H, Maruyama K, Oya Y, Ogawa M, Nonaka I, Kawai M.
Summary at: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=11257793&dopt=Abstract
It seems that the causes, and therefore the treatment can vary. It
can be due to mechanical damage to the muscle, it may occur because of
inflammation of the muscle, or to a destruction of muscle which has no
obvious cause. Another article describes two patients. One of them
showed a progressive weakness of the muscle, but later stabilised. A
biopsy showed destruction of the muscle tissue, but no inflammation,
and he did not respond to corticosteroids. The other patient did show
signs of muscle inflammation, and did respond to corticosteroids.
Journal Clinical Neuroscience 2000 July, vol 7 (no 4) p. 334-336
Myopathic dropped head syndrome: a syndrome of mixed aetiology.
Goh KJ, Wong KT, Tan CT.
Division of Neurology, Department of Medicine, University of Malaya,
Kuala Lumpur, Malaysia
Summary at: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10938615&dopt=Abstract
The question of whether or not the condition is caused by muscle
inflammation seems to be the key to whether medication can be useful.
Inflammation can be treated medicinally. On the other hand, cases
without inflammation are likely to be self-limiting anyway.
“The dropped head syndrome, which occurs in a variety of neuromuscular
disorders, is usually not due to an inflammatory process and generally
either self-limited or nonresponsive to therapy.” However, the
authors go on to describe successful use of immunosuppressive therapy
in one patient who did have inflammation.
Muscle Nerve 1999 June, vol22 (no 6) p.769-771
Focal, steroid responsive myositis causing dropped head syndrome.
Biran I, Cohen O, Diment J, Peyser A, Bahnof R, Steiner I.
Department of Neurology, Hadassah University Medical Center,
Jerusalem, Israel.
Summary: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=10366232&dopt=Abstract
“When a diagnosis cannot be established, it has been considered a
manifestation of a restricted noninflammatory myopathy. A 44-year-old
man presenting with dropped head and subsequent severe limb weakness
did not respond to a first trial of corticosteroids but responded well
to a subsequent prolonged trial of azathioprine followed by
prednisone… … While mechanical damage to overstretched cervical
paraspinal muscles is thought to preclude a response to treatment,
cases of unexplained dropped head syndrome may merit consideration of
prolonged immunosuppressant treatment.”
Muscle Nerve 1999 Jan. vol 22 (no 1) p. 115-118
The dropped head plus syndrome: quantitation of response to
corticosteroids.
Rose MR, Levin KH, Griggs RC.
Department of Neurology, University of Rochester Medical Center, New
York 14642, USA.
Summary:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=9883866&dopt=Abstract
Intensive physiotherapy was found useful in one case: “The dropped
head syndrome: rehabilitation of cervical focal myositis” by Roy
Bahnof Disability and Rehabilitation Volume: 21 Number: 12 Page:
563 – 565
Summary: http://fiordiliji.catchword.com/vl=16661597/cl=15/nw=1/rpsv/cgi-bin/linker?ini=tandf&reqidx=/catchword/tandf/09638288/v21n12/s5/p563
Here is an interesting article by someone who had seen the condition
years before it was first described in the literature (which was in
1992)
“Aging Begins at 30: Finding Name of Mysterious Disease is Gratifying.
Dropped Head Syndrome”
by Ian M. Smith, M.D.
http://www.vh.org/Patients/IHB/IntMed/ABA30/1998/9-17-98Droppedhead.html
Dr Smith emphasizes that the original patients with the classic,
non-inflammatory syndrome were followed for years and found not to
develop any further related problems.
Search strategy: On Medline at PubMed
(http://www.ncbi.nlm.nih.gov/entrez/query.fcgi)
1. neck extensor muscle myopathy 2. dropped head
On Google 1. “neck extensor muscle myopathy” (no hits) 2. “dropped
head” 3 “neck extensor myopathy” |
