The writer examined the pressure, specific gravity, pH., quantity of sugar and protain, the number of blood celles, leper bacilli, colloidal reaction and immune reaction of the cerebrospinal fluid in 31 leprous cases (lepra nodosa 20, lepra ner- vorum 11). The immune reaction was compared with that of the serum in the same patients. The following results were obtained.
(1) The pressure was 55-140mm. in a side position and 210-390 in sitting position, the specific gravity 1006-1010, pH 7, 6-8, 6. The above mentioned results were the same as those in normal conditions of healthy bodies, and there was no marked difference between lepra nodosa and nervorum.
(2) The quantity of sugar usually increased in all leper patients. The increase was more or less in correspondence with the seriousness of the disease. The content of sugar in leprous spinal fluid was 0, 089g/dl-0, 2g/dl in average 0, 122g/dl.
(3) The protain of leperous spinal fluids was more or less influenced by the condition and state of the disease of lepra nodosa and no increase was noticed in lepra nervorum.
(4) The number of blood cells in spinal fluids increased a little in serious cases of lepra nodosa. Leper bacilli could not be found even in one of 31 cases.
(5) The globrin reaction appeared positive in 48% and these were all serious lepra nodosa or nervorum with the complication of syphilis.
(6) Goldsole. reaction appeared in 16 cases positive (57%). Patients who had positive reaction of syphilis, globrin and leper were also found positive in the gold- sole reaction. An intensive positiveness of the goldsole reaction could not be found especially in lepers.
(7) In two cases of lepra nervorum in which the Murata's reaction with the spinal fluid was positive, there appeared negative reaction with the serum. The S. G. R. was positive and the W. R. positive in 19% with the spinal fluid. While syphilitic reactions were found positive with the spinal fluid, they were almost negative with the serum.
(8) An alcoholic extract of leprous nodules could also be used as an antigen in carrying out syphilitic and leprous reaction with the spinal fluid, the precipita- tion reaction positive in 19% and the binding reaction positive in 52%. A liquid emulsion of leprous nodules or tubercle bacilli was suitable as a pure antigen for leprous reactions with the spinal fluid. (32% positive in complement binding reac- tion). The positive cases of leprous reactions with the spinal fluid were also posi- tive with the serum.
(9) All positive reactions with leprous spinal fluids were generally weaker than those with the serum and positive cases of the spinal fluid were very fewer than those of the serum. We could not assume that antibodies could pass from the serum into the spinal fluid under an ordinary condition.
(10) It was very interesting to notice that in leprous reactions of the spinal fluid or serum an antigen from cultured nodules was more effective than that from excised leprous nodules. From cultured nodules numerous lever bacilli could be cultured successfully.

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　Cerebrospinal fluid leakage is associated with several symptoms, such as headache, nausea, photophobia, diplopia and lassitude. In our practice, dizziness is a frequent presenting symptom in patients with this disorder. However, the mechanism and features of dizziness are unclear.
　In this report, we examined two patients with cerebrospinal fluid leakage with dizziness, exhibiting in particular flutter-like oscillations. Ocular flutter is characterized by intermittent bursts of horizontal saccades lacking an intersaccadic interval. Although the precise mechanisms are unknown, ocular flutter is often observed in cerebellar or brainstem lesions. In patients such as ours, ocular flutter may indicates that a central nervous system disorder may be present in cerebrospinal fluid leakage with dizziness.

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国際頭痛分類では第1版（1988年）から低髄液圧，あるいは低髄圧性頭痛が示されている．第3版（2013年β版，2018年）では典型的起立性頭痛で明らかな原因のない患者において，体位性頻脈症候群を除外した後に，自家血腰椎硬膜外注入療法を施行することは臨床診療において理にかなっていると記載された．2007年に

減少症ガイドライン2007，2011年に漏出症の画像診断判定基準・画像診断基準，2019年に漏出症診療指針が出された．頭痛の診療ガイドライン2021に低髄液圧による頭痛はどのように診断し治療するかという項目が取り上げられた．減少症/漏出症を低髄液圧による頭痛としてガイドラインに記載され低髄液圧および／または漏出に続発する起立性頭痛を特徴とする臨床状態と定義して，診断・治療を進めていく道筋が示されている．

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Postural, tension-type headache is the most prominent symptom of cerebrospinal fluid leakage. However, in otoneurological practice, dizziness and not headache is the most frequent presenting symptom in patients with this disorder. Affected patients usually describe their dizziness as a floating sensation. Rotatory vertigo occurs quite rarely. Their complaint is substantiated by high degrees of postural unsteadiness. Some patients cannot stand still even with their eyes open. They stumble and totter while walking. In contrast to such high degrees of postural ataxia, oculomotor disorders such as nystagmus, saccadic pursuit, and decrease of oculomotor nystagmus are not seen. As with other symptoms, dizziness and postural unsteadiness are influenced by the weather, and get worse when the weather is bad. The authors speculate that unsteadiness in both peripheral and central nervous systems involved in spatial orientation (integration of visual, vestibular, and proprioceptive input) underlies the dizziness, postural unsteadiness, and a variety of other symptoms of cerebrospinal fluid leakage.

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Orthostatic headache is a key symptom of cerebrospinal fluid leakage. Other features may include neck pain, interscapular pain, nausea, vomiting, dizziness, tinnitus, and blurred vision. Various psychological or mental symptoms such as depression, forgetfulness, and a decreased power of concentration may also occur in this syndrome. When these symptoms are prominent or precede the onset of other symptoms, the clinical picture of this syndrome becomes more complicated, leading to misdiagnosis or long-term neglect. Of note, patients with cerebrospinal fluid leakage who visit an otolaryngological or oto-neurological clinic usually have atypical manifestations. In the vast majority of our patients with this syndrome, the presenting symptom is dizziness, not headache. The presenting symptom of the 30-year-old female patient who is the subject of this report was also dizziness. The patient's initial symptom was a stomachache; however, medical therapy was ineffective, and endoscopic examination of the stomach revealed no abnormalities. The stomachache was diagnosed as resulting from psychological or mental causes. Other manifestations also indicated that the patient suffered from mental depression. Antidepressants and sleeping pills were prescribed, but no improvement was noted. Dizziness, unsteadiness, nausea, and loss of concentration occurred insidiously and worsened after the patient hit her occiput strongly against a wall during an attack of hyperventilation. The patient also complained of headache. The patient was referred to our clinic by a local otolaryngologist. At the time of the patient's initial visit, a slight unsteadiness was noted while the patient stood and walked, but other vestibular functions were normal. A neurological examination did not show any abnormalities. Psychological testing confirmed the presence of depression. The patient, presumably because of her depression, was dispirited and never discussed her symptoms voluntarily. However, a detailed history revealed that the patient had an orthostatic headache, paresthesia in both hands, blurred vision, and forgetfulness. Several episodes of falls at a pool-side occurring as early as during elementary school were also noted. The diagnosis of cerebrospinal fluid leakage was confirmed by brain MRI and ¹¹¹In-cisternography examinations. An epidural blood patch was performed three times. The patient recovered from her depression; although a mild headache and nausea persisted, all the patient's other symptoms, including dizziness and unsteadiness, improved dramatically. The patient returned to work.

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脊髄液減少症は，

（髄液） が持続的ないし断続的に漏出することによってが減少し，さまざまな症状を呈する疾患である。減少症は，漏出症としてブラッドパッチ治療が保険適応認められたが，診断，治療においてまだまだ問題点の多い疾患である。今回，我々は，2017年4月から2018年6月に当院にて検査入院を行った減少症患者19人のうち漢方薬を使用した患者について検討した。19名中17名にブラッドパッチ治療を行った。そのうち12名に漢方薬を投与した。使用した漢方薬としては，五苓散9名，葛根湯6名，桂枝加朮附湯3名，補中益気湯2名であった。代表症例を提示し考察する。

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減少症の診断の第一歩は，病歴の詳細な聴取である．特に片頭痛，緊張型頭痛，薬物乱用頭痛をベースに減少症を発症してきた場合には，複数の頭痛について根気強く病歴を聞いて，解きほぐして，ベースにある頭痛とともに新たに発症した減少症（通常，鎮痛薬が効かない慢性連日性頭痛，起立性頭痛）を病歴で疑う必要がある．起立性頭痛を呈する体位性頻脈症候群の鑑別でも病歴は重要である．減少症の発症初期は安静だが，体位性頻脈症候群では可能な限り安静を避けるようにするといった対応が必要であり，2つの疾患の鑑別は重要である．減少症の経過中に体位性頻脈症候群に変わってくる症例への対応も重要である．

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　硬膜外自家血注入療法が奏功した外転神経麻痺を伴う

漏出症の1例を報告する．症例は40 歳，女性．嘔吐を伴う起立性頭痛で近医を受診し，片頭痛と診断されたが，その 3 週間後に複視が出現した．頭部造影MRIで硬膜下腔のびまん性拡大，びまん性硬膜造影所見を認めた．低髄液圧症を含めた漏出症の疑いで精査・加療目的で当院脳神経外科へ紹介され，当科受診となった．視力は右（1.0×－18.00D＝Cyl－1.00D Ax180°），左（1.0×－19.00D），眼圧は右13 mmHg，左15 mmHg，前眼部・中間透光体，眼底に異常所見はなかった．眼位検査では遠見25 prism diopter（PD）の内斜視，近見は16PDの内斜視，Hess赤緑試験で右眼の外転制限がみられた．CTミエログラフィーで髄液漏出像がみられ，漏出症と低髄液圧症に伴う外転神経麻痺と診断された．初診時から2週間後，頭痛は消失するも外転神経麻痺が残存したため，初診時から1か月後に硬膜外自家血注入療法が行われた．治療1か月後，複視は消失，眼位は遠見・近見ともに4PDの内斜位，右眼の外転制限も改善した．起立性頭痛を伴う外転神経麻痺をみた場合には，漏出症も念頭に置く必要があり，その治療には硬膜外自家血注入療法が有用であると考えられた．

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Herein, we report two cases of cerebrospinal fluid leakage in children that we encountered at our hospital. Both presented with hearing loss.

Case 1 also suffered from blurred vision and photophobia. He exhibited a series of problem behaviors at school and violent behavior at home. These behaviors had become pronounced after a traffic accident. Ophthalmological testing using an autorefractor revealed the presence of hypertonia of the ciliary muscle and unstable refraction in both eyes. After 4 sessions of treatment with an epidural autologous blood patch (BP), the symptoms improved. The findings in this case suggest that hypertonia of the ciliary muscle and unstable refraction causing visual impairment can occur in cases of cerebrospinal fluid leakage. His abnormal behavior was the result of the abnormalities of the eyes and ears.

Case 2 had chronic dizziness in addition to the hearing loss. The dizziness and abnormalities in the stabilometer test improved a long time after BP treatment. However, the hearing impairment never complately improved.

The interval from the traffic accident to the correct diagnosis was 3 years 9 months in Case 1, and from the initial examination to the correct diagnosis was 3 years 10 months in Case 2. Patients often consult the ENT clinic due to such symptoms as headache, dizziness, hearing loss, tinnitus, and/or visual impairment. We should always bear in mind the possibility of cerebrospinal fluid leakage in such cases. Especially, children with this condition may not be able to precisely express their mental and physical distress, and may exhibit problem behaviors or strange behaviors. Therefore, it is important to obtain a careful history.

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Herein, we report two cases of cerebrospinal fluid leakage in children that we encountered at our hospital. Both presented with hearing loss.

Case 1 also suffered from blurred vision and photophobia. He exhibited a series of problem behaviors at school and violent behavior at home. These behaviors had become pronounced after a traffic accident. Ophthalmological testing using an autorefractor revealed the presence of hypertonia of the ciliary muscle and unstable refraction in both eyes. After 4 sessions of treatment with an epidural autologous blood patch (BP), the symptoms improved. The findings in this case suggest that hypertonia of the ciliary muscle and unstable refraction causing visual impairment can occur in cases of cerebrospinal fluid leakage. His abnormal behavior was the result of the abnormalities of the eyes and ears.

Case 2 had chronic dizziness in addition to the hearing loss. The dizziness and abnormalities in the stabilometer test improved a long time after BP treatment. However, the hearing impairment never complately improved.

The interval from the traffic accident to the correct diagnosis was 3 years 9 months in Case 1, and from the initial examination to the correct diagnosis was 3 years 10 months in Case 2. Patients often consult the ENT clinic due to such symptoms as headache, dizziness, hearing loss, tinnitus, and/or visual impairment. We should always bear in mind the possibility of cerebrospinal fluid leakage in such cases. Especially, children with this condition may not be able to precisely express their mental and physical distress, and may exhibit problem behaviors or strange behaviors. Therefore, it is important to obtain a careful history.

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The most prominent symptom of spinal cerebrospinal fluid (CSF) leakage is an orthostatic, tension-type headache. Other well-known symptoms include nausea, vomiting, photophobia, diplopia, depression, and amnesia. The authors address other commonly encountered symptoms such as dizziness, hearing disturbances, cerebrospinal fluid rhinorrhea, and gustatory and olfactory disturbances.
The dizziness experienced in this disorder is essentially characterized as a kind of “floating sensation” or “walking on the clouds” and is associated with a high degree of unsteadiness. A considerable percentage of patients cannot remain standing even when their eyes are open; to-and-fro perturbations are particularly prominent in these patients. Rotatory vertigo attacks can occur in a small portion of patients, but usually only during the early stages of the disease. As with the headache, dizziness is aggravated by an upright or standing position. The weather also influences the intensity of both the headaches and dizziness: both are exacerbated when the atmospheric pressure is low or is falling rapidly. Water intake and/or the drip infusion of a physiological salt solution may temporarily improve dizziness and other symptoms.
The usefulness of MRI for diagnosing spinal cerebrospinal fluid leakage is limited. MRI findings are equivocal in most cases; therefore, the authors perform ¹¹¹In-DTPA scintigraphy for each patient in whom this disorder is suspected.
The first choice of treatment for this disorder is bed rest and water intake and/or drip infusion. When these treatments are ineffective, an epidural autologous blood patch is attempted. However, not all patients are cured by this procedure, and dizziness, hearing, and/or tinnitus may worsen after treatment. In some patients, an exploratory tympanotomy is required to rule out associated or treatment-induced perilymphatic fistula.
In conclusion, spinal CSF leakage is not a rare disorder, and because this disorder presents with an extremely wide spectrum of symptoms, all physicians in any field of specialization may encounter a patient with this disorder. All physicians should keep this disorder in mind.

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小児期の

漏出症はまれではない．典型的な特発性低髄液圧症候群のような明白な起立性頭痛，MRI所見を呈することは少なく，頭痛，立ちくらみ，起立性低血圧，起立性頻脈症候群，睡眠覚醒障害などのために就学，日常生活に支障がある場合，本症を疑う必要がある．脊髄MRI（脂肪抑制T₂強調画像）が診断に有用であり，上部～中部胸椎レベルの硬膜外高信号が特徴的である．硬膜外ブラッドパッチ治療は安全かつ有効な治療である．硬膜外持続注入は，診断的治療，さらには患者～家族に治療必要性の理解を促す手段として有効である．

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減少症は，従来，低髄液圧症候群と呼ばれていた疾患とほぼ同一の病態の疾患である．その病態がの減少に起因すると考えられるため，減少症がより適切な疾患名となっている．の減少により，頭痛，頸部痛，めまい，耳鳴，視機能障害，倦怠・易疲労感など様々な症状を呈する疾患と定義される．本疾患の診断に有用な画像診断法には，頭部MRI（magnetic resonance imaging）やRI脳槽・脊髄液腔シンチグラムが挙げられ，治療は安静臥床，輸液による保存的治療と硬膜外自家血注入療法が一般的である．減少症は未だ医療関係者の間でも十分に認識されているとは言い難く，しばしば誤った診断，治療がなされている．現在，厚生労働省の班会議「減少症の診断・治療の確立に関する調査研究」で，減少症の診断・治療指針（ガイドライン）の作成を目標とした研究が進行中であり，その調査結果が待たれるところである．

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患者は生来健康な26歳，女性．職業はキャビンアテンダント．勤務中に乗客の重い荷物を全身の力で頭上の棚に持ち上げ，その夜より強い頭痛と嘔気が生じた．頭痛や嘔気は立位や夕方に増強し，臥位で改善した．computed tomography（CT）脊髄造影で髄液漏出が認められ，減少症と診断された．治療として硬膜外生理食塩液持続注入を行い，症状・activities of daily living（ADL）ともに改善がみられた．合併症が多いと考えられる硬膜外自家血注入を行わず，安全かつ効果的に治療することができた．また，非外傷性の減少症は発症契機が不明なことが多いが，本症例では重い荷物を頭上に持ち上げるようなストレッチ運動が契機となった可能性が高いと考えられた．

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減少症の診断に脳脊髄腔シンチグラフィが有用であった2例を報告する。症例は，難治性の頭痛を主訴に来院した31歳の男性と34歳の女性である。2例とも頭部造影MRI検査で異常所見を認めなかった。減少症が疑われたため，脳脊髄腔シンチグラフィを行った。1例は，膀胱の早期描出像，脳脊髄腔内RI残存率の減少を認めた。他の1例は，漏出像，膀胱の早期描出像，脳脊髄腔内RI残存率の減少がみられ，2例とも減少症と診断された。臨床的に減少症が疑われるときは，頭部造影MRI所見が正常であっても脳脊髄腔シンチグラフィは，有用な検査と考える。

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減少症と心身症には3つのかかわりがある.第一に心身症と診断されている減少症患者が少なくないこと,第二に減少症の症状に心身症的な症状が含まれていること,第三に減少症の治療には心身症的な治療が必要であること,である.が減少することにより頭痛,めまい,視覚障害,記憶力低下,倦怠など多彩な症状が持続する疾患である.原因が不明の特発性と外傷性があるが,心身症的症状を呈するのは外傷性に多い.診断は体位により変化する多彩な症状とMRIや放射性同位元素(RI)脳槽シンチグラフィー・CTミエログラフィーなどの画像診断によってなされる.治療は減少した量を増やすことであるが,漏れを止めるためには自家血を脊髄硬膜外に注入するブラッドパッチ治療が効果的である.

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