― 112 ―Dementia Japan 30 : 112-115, 2016

Original

A case of Hashimoto’s encephalopathy precededby recurrent episodes of neurological disturbances
Chizuko Toyoda1), Tadashi Umehara2) and Hisayoshi Oka2)

logical disturbances should be evaluated.

Abstract

Key words : Hashimoto’s encephalopathy, anti-NH2terminal of alpha-enolase (NAE) antibody, disturbanceA 72-year-old woman with autoimmune hepatitis,of consciousness, autoimmune diseaseHashimoto’s disease, diabetes mellitus and liver cir-Introduction

rhosis was admitted to our hospital with disturbance ofconsciousness.

Her medical state was stabilized bymedications.

The high level of serum anti-thyroid

Hashimoto’s encephalopathy is an autoimmune dis-antibodies suggested Hashimoto’s encephalopathy,ease that shows positivity for anti-thyroid antibodies

thus steroid therapy was started.

and various clinical manifestations.

The patient’sPatients withserum anti- NH 2-terminal of alpha-enolase (NAE)

Hashimoto’s encephalopathy benefit from immuno-

antibody test was strongly positive.

modulatory therapy such as steroids.

After predniso-

We hereinlone therapy, the patient’s consciousness level gradu-

report a case of Hashimoto’s encephalopathy withally improved, and she was discharged on hospital day

rapid progression after recurrent episodes of neurolog-

170 after rehabilitation.

ical disturbances and disturbance of consciousness.

This case suggests that due

to the various patterns of onset of Hashimoto’s

Case Reportencephalopathy, anti-thyroid antibodies in cases ofunidentified disturbance of consciousness and neuro-Since the age of 51, the patient had been treated for
繰り返す神経学的異常所見と共に症状が進行した橋本脳症の 1 例淳2），岡尚省2）
豊田千純子1），梅原1）
Department of Neurology, The Jikei University School of Medicine（3-25-8 Nishishinbashi, Minato-ku, Tokyo 105-8461, Japan）
東京慈恵会医科大学神経内科［〒 105-8461 東京都港区西新橋3-25-8］2）
Department of Neurology, Daisan Hospital, The Jikei University
School of Medicine（4-11-1 Izumihonchou, Komae-shi, Tokyo 2018601, Japan）
東京慈恵会医科大学附属第三病院神経内科［〒 201-8601 東京
都狛江市和泉本町 4-11-1］

autoimmune hepatitis, Hashimoto’s disease, diabetes
mellitus, and liver cirrhosis with an unidentified age ofonset.

Her medical condition was stabilized by treat-ment with prednisolone (5 mg/day) and levothyroxine(50 μg/day).

She showed difficulties in standing up

from January 2012, and urinary and bowel incontinenceand slow, involuntary movement of the fingers of both― 113 ―A case of Hashimoto’s encephalopathy preceded by recurrent episodes of neurological disturbances
Table 1.

The laboratory DataWBC

9,100/μL

Na

131 mEq/L

RF

34.4 IU/ml

RBC

433×104/μL

K

3.8 mEq/L

ANA

92.5

Hb

13.8 g/dL

Cl

98 mEq/L

MPO-ANCA

Ht

41.6%

CRP

anti-SS-AAb>256xPlt8.4×104 /μL

NH3Glu3.0 mg/dL
18 μg/dL

1,530 IU/ml

133 mg/dL

anti-TGAb
anti-TPOAb

HbA1c

6.7%

TSH
fT3fT41.38 μIU/mL
2.42 pg/mL
1.25 ng/dL

AST
ALT
LDHγGT
TPAlb
BUN
Cr

42 U/L38 IU/L
138 IU/L
43 IU/L
7.6 g/dL
2.9 g/dL
11 mg/dL
0.78 mg/dL

19.9 U/ml

71 IU/ml

RF : rheumatoid factor, ANA : anti-nuclear antibody, MPO-ANCA : myeloperoxidase anti-neutrophil cytoplasmic antibody, anti-SS-A Ab : anti-Sjogren’s syndrome A antibody, anti-TG Ab : anti-thyroglobulin antibody, anti-TPO Ab : anti-thyroid peroxidase antibody

hands developed in August 2012, and she had began

On hospital day 2, the electroencephalography

experiencing daytime sleepiness from December(EEG) showed a slow  wave basic rhythm (8 Hz) with

2012 ; in mid-December, she was found lying on theθ and β waves without epileptic discharges.

floor with the fingers of both hands woven together,magnetic resonance imaging (MRI) showed lacunar

and was admitted to the hospital with disturbance ofinfarcts and bilateral deep white matter lesions (Figureconsciousness.

1).

On hospital day 1, she was unconscious with a blood

Brain

Brain single photon emission computed tomogra-phy (SPECT) showed no areas with hypo-perfusion.

pressure of 162/92 mmHg, and temperature of 37.8°C.

Anticonvulsant therapy was ineffective, and her

She demonstrated neck stiffness, a positive Kernig

state of consciousness gradually worsened and fluc-sign, and tonic arms.

tuated.

We observed no signs of meningoencephali-

The patient’s laboratory data are shown in Table 1.

tis, hepatic encephalopathy, or symptomatic epilepsy.

Mild liver damage was found without NH3 elevation.

These findings together with the elevated level of anti-Plasma glucose levels and thyroid function were

thyroid antibodies suggested Hashimoto’s encephalop-normal.

Autoimmune antibody test results were as

athy, and on hospital day 9, we started the patient onfollows : rheumatoid factor, 34.4 IU/mL ; anti-nuclear

methyl-prednisolone pulse therapy and 40 mg/day oral

antibody, positive ; myeloperoxidase anti-neutrophilprednisolone.

cytoplasmic antibody, 19.9 U/mL ; anti Sjögren’s syn-level of consciousness gradually improved, and thedrome A antibody, >256x ; anti thyroglobulin (TG)anti-thyroid antibody titers decreased by half within

antibody, 1,530 IU/mL ; and anti-thyroid peroxidase

the first 2 weeks.

--(TPO) antibody, 71 IU/mL.

Notably, her anti thyroid-

After the steroid therapy, the patient’s

We gradually decreased the pred-

nisolone dose by 5 mg/week to 10 mg/day on hospital

antibody titers were high, and her anti NH2 terminalday 32.

of alpha enolase (NAE) antibody titer was strongly

charged home on hospital day 170 (Figure 2).

---positive.

A lumbar puncture revealed clear cerebro-

spinal fluid with a normal cell count and protein level.

After rehabilitation, the patient was dis-― 114 ―Dementia Japan Vol. 30 No. 1 January 2016

Figure 1.

 luid-attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI)F
showed high intensity areas in the bilateral deep white matter and the periventricular and pons areas.

Figure 2.

 imeline of clinical events following admission. After recurrent episodes ofT
neurological disturbances, the patient was admitted to our hospital due to disturbance of consciousness. Methyl-prednisolone pulse therapy and oral prednisolone therapy were effective. The anti-thyroglobulin (TG) antibody titers
decreased by one-fifth, and anti-thyroid peroxidase (TPO) antibody titers
decreased by half. After tapering of the steroid and rehabilitation, the patient
was discharged home.

brain MRI or SPECT before and after therapy.

Discussion

Onlaboratory examinations, the high titers of anti TG-antibody and anti-NAE antibody were characteristic
In this patient, rapid progression of disturbance offeatures.

For the diagnosis of Hashimoto’s encepha-consciousness with fever developed after 1 year with alopathy, we used the Shaw PJ et al. (1991) criteria,fluctuating state.

which include psychosis, anti-thyroid antibody positiv-

We found no significant change on

― 115 ―A case of Hashimoto’s encephalopathy preceded by recurrent episodes of neurological disturbances

ity, and effectiveness of steroid therapy.

Perschen-turbances and disturbance of consciousness is uniden-

Rosin R et al. added findings from cerebrospinal fluid,tified, because Hashimoto’s encephalopathy has

EEG and brain MRI to these criteria (1999).

various patterns of onset.

How-ever these criteria are not adequate for Japanese

Acknowledgment

patients in terms of the disease specificity of anti-thyroid antibody.

Yoneda et al. discovered the diagnostic

significance of anti-NAE antibody and reported its highWe thank Dr. Akiko Matsunaga from the medical

prevalence in Hashimoto’s encephalopathy (Fujii et al.,department of Fukui University and Dr. Makoto Yoneda

2005 ; Yoneda et al., 2007).

from Fukui Prefectual University for their help with

Recently, Yoneda pro-

posed new criteria, which include positivity for anti-the measurement of anti-NAE antibody.

NAE antibody (2013).
In a study of 80 patients with Hashimoto’s encepha-References

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52 : 1240 1242 (in Japanese, Abstract in English)

Hashimoto’s encephalopathy is a disorderthat involves immune pathogenic mechanisms, but theroles of anti-thyroid antibodies and anti-NAE antibodies have not been fully elucidated (Schiess et al., 2008).
This case suggests that we should evaluate antithyroid antibodies when the cause of neurological dis-Manifesta-

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