Makiko Kaga - Landau-Kleffner Syndrome and Central Auditory Disorders in Children

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The author has long been fascinated by the mysterious symptoms of LandauKleffner syndrome (LKS). LKS was named in honor of Drs. William Landau and Frank Kleffner. It was a chance encounter with patients with LKS who were the patients of the late Dr. Masaki Suzuki (19301976). Dr. Suzuki was a child neurologist who was interested in neuropsychology and neuropsychiatry in children. He was one of my mentors in my professional life as a child neurologist. However, he was my teacher only for a year and a half, and he passed away all of a sudden at 46 years of age.

When I first met children with LKS, their diagnosis was acquired aphasia in children with markedly epileptic abnormal electroencephalogram. Their sequential symptoms of aphasia, auditory agnosia, and word deafness without apparent organic brain lesions simply surprised me. Usually these symptoms of higher cortical dysfunction cannot occur with responsible brain lesions. During their long-term follow-up, most patients fully recovered without apparent handicaps. They spend their lives as the typically developed children/adults. I was again surprised that almost all patients lost their musical ability and such a state continued thereafter even after they became free of auditory verbal symptoms. Moreover, there are some children with incomplete or scarce recovery. I have known and have been impressed by a lady who had a severe sequela of auditory agnosia for more than 50 years. Her efforts to overcome and at the same time to live together with her handicaps is worthy of special mention.

In this book, I tried to show the clinical features of LKS mainly from the viewpoint of auditory agnosia which is the central auditory dysfunction.

Thus, the book is for auditory agnosia and related central auditory dysfunction in children. Part I of this book is just for LKS which is a disease of functional abnormality and was written by Makiko Kaga.

Part II of this book is for the diseases which can manifest central auditory dysfunction due to organic brain lesions. This part was divided up between Makiko Kaga and Kimitaka Kaga. Kimitaka Kaga is an otorhinolaryngologist and is deeply engaged in both peripheral and central auditory dysfunctions in all generations.

We plan to put this book to help diagnosing and treating the rare condition of auditory agnosia in children induced by LKS and other diseases which may develop central auditory dysfunction and are difficult to diagnose. These patients need to be treated and supported for a rather long time.

I appreciate Masaki Suzuki, MD, PhD (19301976); Isabelle Rapin, MD (19272017); Thiery Deonna, MD; Yoshisato Tanaka, MD, PhD; and Masataka Arima, MD, PhD, for inspiring me about LKS, language development, hearing impairment, neurodevelopmental disorders, and childhood higher cortical functions.

I acknowledge Drs. Osamu Kanazawa, MD, PhD; Shunichi Kato, MD, PhD; Koji Kato, MD, PhD; Hirokazu Oguni, MD, PhD; Norio Sakuragawa, MD, PhD; Masayuki Shimohira, MD, PhD; and Nobuyuki Shimozawa, MD, PhD, for referring patients.

We thank our colleagues, Masumi Inagaki, MD, PhD; Atsuko Gunji, PhD; Kotoe Sakihara, PhD; Wakana Furushima, MD, PhD; Seiko Suzuki, MD, PhD; Toshihiro Horiguchi, PhD; Yoshimi S Kaga, MD, PhD; Takayuki Hatori, MD, PhD; Shingo Oana, MD, PhD; Kyoko Sasaki MD; Naomi Kokubo, PhD; Tatsuyuki Ohto, MD, PhD; Miho Nakamura, MD, PhD; Tatsuya Koeda, MD, PhD; Kenji Sugai, MD, PhD; Takashi Saito, MD, PhD; Hitomi Noguchi, MD, PhD; Mitsuko Shindo, PhD; Masako Nakamura, MS; Reiko Ohta, BS; and Kayoko Sekiguchi, BS.

This book contains the results of Research Grant of Encouragement of Scientists in 2009 from the Ministry of Health, Labor and Welfare, Japan, Present a real picture of LandauKleffner syndrome (Chief scientist: Makiko Kaga).

In this research project, we thank the Directors of Pediatrics in all Japanese Hospitals which had pediatric departments, councilors of the Japanese Society of Child Neurology, and councilors of the Japan Epilepsy Society for cooperating with the nationwide survey of LandauKleffner syndrome.