Geriatr Gerontol Int 2007; 7: 195–197

CASE REPORT

Successful treatment of
spinocerebellar ataxia 6 with
medicinal herbs
Tetsuro Okabe,1 Michio Fujisawa,2 Takasi Sekiya,1 Yaeko Ichikawa3 and
Jun Goto3
1
Department of Integrated Traditional Medicine, Graduate School of Medicine, 2Health Service Center,
and 3Department of Neurology, University of Tokyo, Hongo, Tokyo, Japan

A case of familial spinocerebellar ataxia 6 with typical symptoms is presented. A 60-year-

old Japanese female suffered from gait disturbance, ataxia and dizziness. Head magnetic
resonance imaging revealed a typical atrophic image in cerebellum. Genetic tests revealed
an expanded allele of 22 CAG repeats at the spinocerebellar ataxia type 6 locus. She was
diagnosed with spinocerebellar ataxia 6. Her mother was also diagnosed with the same
disease. A mixture of 18 medicinal herbs (modified Zhengan Xifeng Tang) was given
according to the differential diagnosis based on the guidelines of traditional Chinese
medicine. All of the symptoms were remarkably improved after 60 days of the herbal
treatment. One year after discontinuation of the treatment, she complained of gait ataxia.
She was treated with the modified Zhengan Xifeng Tang for 60 days. Gait ataxia was
markedly improved by the second treatment. Fifteen months after discontinuation of the
second treatment, she complained of gait ataxia again. The same remedy was given for
60 days. Gait ataxia was remarkably reduced again. The results may imply therapeutic
potential of the medicinal herbs for spinocerebellar ataxia 6.

Keywords: genetics, herbs, nervous system disease, therapy, traditional Chinese medicine.

Introduction Case report

Spinocerebellar ataxias (SCA) are a clinically and geneti-
cally heterogeneous group of neurodegenerative disor-
ders. Although transient improvement of ataxia with
thyrotropin-releasing hormone, branched-chain amino
acid, or zolpidem have been reported,1–3 we have no
effective therapeutic tools against these degenerative
diseases. We report here a case of familiar spinocerebel-
lar ataxia 64 with typical symptoms, which were
improved after administration of a mixture of traditional
medicinal herbs (modified Zhengan Xifeng Tang).5
Accepted for publication 25 November 2005.
Correspondence: Dr Tetsuro Okabe MD, Department of Inte-
grated Traditional Medicine, Graduate School of Medicine, Uni-
versity of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655,
Japan. Email: okabe-tky@umin.ac.jp
A 60-year-old Japanese female suffering from tituba-
tion, muscle rigidity, loss of balance and dizziness was
diagnosed with familial spinocerebellar ataxia by head
magnetic resonance imaging (MRI). Loss of balance had
gradually progressed until she could not walk well by
herself for the past 10 years. Tendon reflexes were not
increased. Mild modification of fluency and slight
slurring were observed. Mild ataxia was found on the
lower extremities but not on the upper extremities.
There were no oculomotor abnormalities, including
nystagmus. Autonomic disorders were not found, To
identify various subtypes SCA, SCA1, dentatorubral-
pallidoluysian atrophy (DRPLA), Machado–Joseph
disease (SCA3/MJD), and SCA6 loci were assessed for
expansion of trinucleotide repeats.6 Analysis of the
SCA6 CAG trinucleotide repeat at the CACNA1A gene
in the patient’s DNA demonstrated an expanded allele
of 22 CAG repeat units.4 She had no significant past

© 2007 Japan Geriatrics Society doi: 10.1111/j.1447-0594.2007.00378.x 兩 195

 T Okabe et al.
Table 1 Ingredients of modified Zhengan Xifeng
Tang (g)
Achyranthes bidentatae Blume root (4)
Fossilia ossis mastodi (smashed) (4)
Concha ostreae (smashed) (3)
Gasrodia elata Bl root (3)
Uncaria rhynchophylla Jacks stem with hooks (3)
Paeonia lactiflora Pall root (4)
Asparagus cochinchinensis Merr root (3)
Rhemannia glutinosa Libousch root (4)
Melia toosendan Sieb et Zucc fruit (3)
Paeonia suffruticosa Andr bark (4)
Gentiana scabra Bge root (3)
Coptis chinensis Franch root (3)
Phellodendron amurense Rupr bark (3)
Salvia miltiorrhiza Bge root (3)
Zizyphus spinosus Hu seed (3)
Magnolia officinalis Rehd et Wils bark (3)
Chaenomeles lagenaria Koidz fruit (3)
Glycyrrhiza uralensis Fisch root (2)
Eighteen ingredients were boiled in 500 mL of water for
30 min. The supernatant was given three times a day
between meals and before sleep.
medical history. Her mother was diagnosed with the
same disease by MRI had suffered from severe gait dis-
turbance and was not able to walk well by herself. A
younger sister of her mother had been also diagnosed
with spinocerebellar degeneration and became
wheelchair-bound 5 years after the diagnosis.
Under the differential diagnosis by traditional
Chinese medicine, a mixture of 18 medicinal herbs (a
modification of Zhengan Xifeng Tang; Uchida
Wakanyaku, Tokyo, Japan) was boiled and the super-
natant was administered three times a day for 60 days
(Table 1), which was expected to stabilize her central
nervous system and is usually used against tremor,
vertigo or ataxia in traditional Chinese medicine.2 All of
the symptoms including ataxia, dizziness and titubation
remarkably improved after 60 days of the treatment. Her
total ataxia score was improved from 20 to 8 on a 100-
point semiquantitative International Cooperative Ataxia
Rating Scale (ICARS).7 To consolidate the effect of the
medicinal herbs, the same remedy was given for another
60 days. One year after discontinuation of the treat-
ment, she complained of gait ataxia. The modified
Zhengan Xifeng Tang was administered for 60 days.
Ataxia was markedly improved after 60 days of the treat-
ment. Fifteen months after discontinuation of the treat-
ment, she complained of gait ataxia again. The modified
Zhengan Xifeng Tang was administered for 60 days.
Ataxia was remarkably reduced again. No adverse effects
were observed. Even after discontinuation of the
therapy, she has been well without any symptom for
196 兩
Figure 1 A representative magnetic resonance imaging of
the brain showing atrophy of the cerebellum.
these 3 months. Head MRI revealed no apparent exac-
erbation after the therapy (Fig. 1).
Discussion
A case of familial SCA6 was successfully treated by a
mixture of 18 medicinal herbs. This may be the first case
report of a successful treatment of familial SCA6. Effec-
tive treatments of the hereditary disorders have not been
developed. All the symptoms dramatically improved
after 60 days of the treatment. While the disease
relapsed 12 months after discontinuation of the treat-
ment, all the symptoms were markedly improved again
by re-administration of the same herbs. The patient did
not have a relapse for another 15 months after discon-
tinuation of the modified Zhengan Xifeng Tang. After
the second relapse, the modified Zhengan Xifeng Tang
was administered for 60 days. The third treatment was
also effective and ataxia was remarkably improved
again.1–3
Recently, an anticonvulsive herb, Gastrodia elata, a
component of our modified Zhengan Xifeng Tang, has
been shown to protect against kainic acid-induced neu-
ronal damages in the mouse hippocampus.8 Magnolia
officinalis, included in the recipe, has been shown to
exert antidepressant and anxiolytic effects in mice.9,10
The ethanol extracts of Paeonia lactiflora root, another
component of the modified Zhengan Xifeng Tang and
its active constituents, gallic acid and methyl gallate,
exhibited a significant free-radical scavenging effect
against 1,1-diphenyl-2-picryl hydrazine radical genera-
tion and had an inhibitory effect on lipid peroxidation.
In addition, they strongly inhibited the hydrogen
peroxide-induced DNA damage in mammalian cells.11
Together with these observations, it is possible to
© 2007 Japan Geriatrics Society
 Spinocerebellar ataxia and medicinal herbs
consider that some herbs in the modified Zhengan
Xifeng Tang exert their long-term effects through the
neuroprotective activities against neuronal degeneration
in the cerebellum. Further investigations are required
on the mechanisms by which the long-term remission
of the genetic disease has been obtained. This line of
therapeutic approach will be the beginning of the study
on the maintenance of the neuronal functions in this
hereditary degenerative disorder.
Acknowledgments
The authors thank Kyoko Hasegawa for aiding in the
preparation of DNA.
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