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- A 60-year-old Japanese female suffering from tituba-
cally heterogeneous group of neurodegenerative disor- tion, muscle rigidity, loss of balance and dizziness was
ders. Although transient improvement of ataxia with diagnosed with familial spinocerebellar ataxia by head
thyrotropin-releasing hormone, branched-chain amino magnetic resonance imaging (MRI). Loss of balance had
acid, or zolpidem have been reported,1–3 we have no gradually progressed until she could not walk well by
effective therapeutic tools against these degenerative herself for the past 10 years. Tendon reflexes were not
diseases. We report here a case of familiar spinocerebel- increased. Mild modification of fluency and slight
lar ataxia 64 with typical symptoms, which were slurring were observed. Mild ataxia was found on the
improved after administration of a mixture of traditional lower extremities but not on the upper extremities.
medicinal herbs (modified Zhengan Xifeng Tang).5 There were no oculomotor abnormalities, including
nystagmus. Autonomic disorders were not found, To
identify various subtypes SCA, SCA1, dentatorubral-
pallidoluysian atrophy (DRPLA), Machado–Joseph
Accepted for publication 25 November 2005. disease (SCA3/MJD), and SCA6 loci were assessed for
expansion of trinucleotide repeats.6 Analysis of the
Correspondence: Dr Tetsuro Okabe MD, Department of Inte-
grated Traditional Medicine, Graduate School of Medicine, Uni- SCA6 CAG trinucleotide repeat at the CACNA1A gene
versity of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, in the patient’s DNA demonstrated an expanded allele
Japan. Email: [email protected] 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) Figure 1 A representative magnetic resonance imaging of
Magnolia officinalis Rehd et Wils bark (3) the brain showing atrophy of the cerebellum.
Chaenomeles lagenaria Koidz fruit (3)
Glycyrrhiza uralensis Fisch root (2)
Eighteen ingredients were boiled in 500 mL of water for these 3 months. Head MRI revealed no apparent exac-
30 min. The supernatant was given three times a day erbation after the therapy (Fig. 1).
between meals and before sleep.
Discussion

medical history. Her mother was diagnosed with the A case of familial SCA6 was successfully treated by a
same disease by MRI had suffered from severe gait dis- mixture of 18 medicinal herbs. This may be the first case
turbance and was not able to walk well by herself. A report of a successful treatment of familial SCA6. Effec-
younger sister of her mother had been also diagnosed tive treatments of the hereditary disorders have not been
with spinocerebellar degeneration and became developed. All the symptoms dramatically improved
wheelchair-bound 5 years after the diagnosis. after 60 days of the treatment. While the disease
Under the differential diagnosis by traditional relapsed 12 months after discontinuation of the treat-
Chinese medicine, a mixture of 18 medicinal herbs (a ment, all the symptoms were markedly improved again
modification of Zhengan Xifeng Tang; Uchida by re-administration of the same herbs. The patient did
Wakanyaku, Tokyo, Japan) was boiled and the super- not have a relapse for another 15 months after discon-
natant was administered three times a day for 60 days tinuation of the modified Zhengan Xifeng Tang. After
(Table 1), which was expected to stabilize her central the second relapse, the modified Zhengan Xifeng Tang
nervous system and is usually used against tremor, was administered for 60 days. The third treatment was
vertigo or ataxia in traditional Chinese medicine.2 All of also effective and ataxia was remarkably improved
the symptoms including ataxia, dizziness and titubation again.1–3
remarkably improved after 60 days of the treatment. Her Recently, an anticonvulsive herb, Gastrodia elata, a
total ataxia score was improved from 20 to 8 on a 100- component of our modified Zhengan Xifeng Tang, has
point semiquantitative International Cooperative Ataxia been shown to protect against kainic acid-induced neu-
Rating Scale (ICARS).7 To consolidate the effect of the ronal damages in the mouse hippocampus.8 Magnolia
medicinal herbs, the same remedy was given for another officinalis, included in the recipe, has been shown to
60 days. One year after discontinuation of the treat- exert antidepressant and anxiolytic effects in mice.9,10
ment, she complained of gait ataxia. The modified The ethanol extracts of Paeonia lactiflora root, another
Zhengan Xifeng Tang was administered for 60 days. component of the modified Zhengan Xifeng Tang and
Ataxia was markedly improved after 60 days of the treat- its active constituents, gallic acid and methyl gallate,
ment. Fifteen months after discontinuation of the treat- exhibited a significant free-radical scavenging effect
ment, she complained of gait ataxia again. The modified against 1,1-diphenyl-2-picryl hydrazine radical genera-
Zhengan Xifeng Tang was administered for 60 days. tion and had an inhibitory effect on lipid peroxidation.
Ataxia was remarkably reduced again. No adverse effects In addition, they strongly inhibited the hydrogen
were observed. Even after discontinuation of the peroxide-induced DNA damage in mammalian cells.11
therapy, she has been well without any symptom for Together with these observations, it is possible to

196 兩 © 2007 Japan Geriatrics Society

 Spinocerebellar ataxia and medicinal herbs

consider that some herbs in the modified Zhengan polyglutamine-dependent expansions in the alpha
Xifeng Tang exert their long-term effects through the 1A-voltage calcium channel. Nat Genet 1997; 15: 62–69.
5 Zhan E. Prescriptions of Traditional Chinese Medicine. Shang-
neuroprotective activities against neuronal degeneration
hai, China: Publishing house of Shanghai college of tradi-
in the cerebellum. Further investigations are required tional Chinese medicine, 1990.
on the mechanisms by which the long-term remission 6 Watanabe H, Tanaka F, Matsumoto M, Doyu M, Ando T,
of the genetic disease has been obtained. This line of Sobue G. Frequency analysis of autosomal dominant cer-
therapeutic approach will be the beginning of the study ebellar ataxias in Japanese patients and clinical caharcter-
ization of spinocerebellar ataxia type 6. Clin Genet 1998; 53:
on the maintenance of the neuronal functions in this
13–19.
hereditary degenerative disorder. 7 Trouillas PT, Takayanagi M, Hallett RD et al. The Ataxia
Neuropharmacology Committee of the World Federation
Acknowledgments of Neurology International Cooperative Ataxia Rating
Scale for pharmacological assessment of the cerebellar syn-
drome. J Neurol Sci 1997; 145: 205–211.
The authors thank Kyoko Hasegawa for aiding in the
8 Kim HJ, Moon KD, Oh SY, Kim SP, Lee SR. Ether fraction
preparation of DNA. of methanol extracts of Gastrodia elata, a traditional
medicinal herb, protects against kainic acid-induced neu-
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