A case of musical agraphia

Damage to the left upper parietal lobule causes pure agraphia. However, we experienced a patient who exhibited musical agraphia following such a lesion after the agraphia improved. The patient was a 53-year-old female piano teacher. After surgery, she exhibited agraphia and musical agraphia. There was no expressive amusia, receptive amusia, aphasia, agnosia or apraxia. Fifteen months post-surgery, when her agraphia had resolved, her abilities to read, write, and copy music were evaluated. She could read and write single notes and musical signs, but her ability to write a melody was seriously impaired. Furthermore, the salient impairment was in writing rhythm rather than pitch. She could copy music, but only slowly. We consider her a case of pure musical agraphia.     

A nonmusician with severe Alzheimer’s dementia learns a new song

The hallmark symptom of Alzheimer’s Dementia (AD) is impaired memory, but memory for familiar music can be preserved. We explored whether a non-musician with severe AD could learn a new song. A 91 year old woman (NC) with severe AD was taught an unfamiliar song. We assessed her delayed song recall (24 hours and 2 weeks), music cognition, two word recall (presented within a familiar song lyric, a famous proverb, or as a word stem completion task), and lyrics and proverb completion. NC’s music cognition (pitch and rhythm perception, recognition of familiar music, completion of lyrics) was relatively preserved. She recalled 0/2 words presented in song lyrics or proverbs, but 2/2 word stems, suggesting intact implicit memory function. She could sing along to the newly learnt song on immediate and delayed recall (24 hours and 2 weeks later), and with intermittent prompting could sing it alone. This is the first detailed study of preserved ability to learn a new song in a non-musician with severe AD, and contributes to observations of relatively preserved musical abilities in people with dementia.     

Case of singing seizure using syllable names

We report a case of a patient with singing seizures, who was able to sing familiar songs by syllable name with no earlier practice. The patient was a 56-year-old musically naive woman who developed singing seizures when she was in her early 20s. She suddenly began singing familiar sacred songs by syllable name, even though she had never practiced the songs using a musical score or had earlier sung them by syllable name. An electroencephalogram showed bilateral low-voltage spikes that were significantly pronounced in the right temporal lobe. Technetium-99m-bicisate ethyl cysteinate dimer single photon emission computed tomography also showed hypoperfusion in the medial right temporal lobe. The right temporal lobe may be involved in singing, and there may be an automatic and unconscious analytical system of music perception that arranges each tone into its syllable name.     

A case of cerebral infarction presenting as retrosplenial amnesia]

We report a 73-year-old right-handed female who presented with an acute amnesic syndrome. On November 18, 1991, she was admitted to a local hospital complaining of sudden-onset vertigo and nausea, but immediately after the admission she developed an amnesic syndrome. On November 27, she was transferred to our hospital for further assessment of her memory disturbance. Neurologically she was normal except for mild right hemianopsia and increased deep tendon reflexes in the extremities. Neuropsychological assessments were performed over 3 weeks. She was always alert, attentive, and cooperative. She had no confabulation. On the Wechsler Adult Intelligence Scale revised (WAIS-R), her total IQ was 110. Frontal, verbal, and perceptual functions and motor performance were normal. She had no signs of a callosal disconnection. Despite these preserved functions, her memory function was obviously disturbed. Several memory betteries showed that her recent memory for both verbal and visual modalities was impaired, while her immediate memory such as digit span was preserved. For remote memory her retrograde episodic memory concerning both personal and public events was almost intact, although she had a profound anterograde amnesia. In particular she recalled her personal information about just-premorbid events in detail. On the other hand, her semantic memory, for example understanding of proverbs, geography, and scientific law, was preserved. Taken together, her procedural memory on learning tasks, such as "Tower of Hanoi" and mirror drawing, was intact. Computed tomography demonstrated a low-density area medial to the trigon of the left ventricle.(ABSTRACT TRUNCATED AT 250 WORDS)     

A clinical course and autopsy results of an 8-year-old severely handicapped girl with marked periventricular leukomalacia]

We reported a clinical course and autopsy results of an 8-year-old severely handicapped girl with marked periventricular leukomalacia. She was well until 3 days prior to first admission in local hospital. Two days prior to admission, she began to vomit. Twelve hours later, she was noted to be lethargic and developed malaise with frequent vomiting. At physical examination on admission, she had frequent fits and her posture was decerebrate rigidity. Consciousness disturbance continued for two weeks. Thereafter, she became severely handicapped with spastic quadriplegia, mental retardation and intractable epilepsy. She was transferred to our hospital one month later. We cared her totally and carefully with our rehabilitation staff, but during her course several rare happening occurred; she suffered from subdural hemorrhage due to hypocupremia and received an operation for the release of contracture of her hips. She died of acute cardio-respiratory failure at 8 years and 5 months of age. Her autopsy findings were characteristic of the damage to an immature brain during development; cactus formation of cerebellar cortex and periventricular leukomalacia.     

Music memory provides access to verbal knowledge in a patient with global amnesia

Abstract

We report on the patient CH, who showed marked impairment of declarative memory in everyday life and standardized memory tests subsequent to herpes simplex encephalltis. However, there was anecdotal evidence for preserved learning capacity for verbal material (song titles) in connection with playing accordion music. Our experiments, tallored to CH's individual accordion repertoire, confirmed strong associations between language (song titles) and music (accordion melodies): CH performed 100% of the melodies correctly after being presented with the respective song titles. Presented with melodies, she recognized 90% of the titles out of several distractors. This good verbal memory success is conspicuous in the light of her otherwise severe memory impairments. To elucidate the phenomenon, we examined additionally the semantic knowledge concerning music-associated verbal material in CH by definition, categorization, reading and naming tasks. If music-associated words belonged to an actually better preserved or relearned vocabulary, we would have expected CH to perform better with music-associated concepts than with comparable, non-music-associated concepts. However, that was not the case. CH's good verbal memory in a musical context may be explained in terms of a material-specific, semantically empty priming effect.     

Piano playing in Alzheimer's disease: Longitudinal study of a single case

Abstract

Preserved musical performance by patients with Alzheimer's disease (AD) has been attributed to an intact system for implicit musical cognition which may be localized in the right hemisphere. To examine this idea more thoroughly, we studied patient ML, a woman with AD who learned to play the piano as a child. Over three yearly evaluations, ML exhibited progressive deterioration in the following domains: language, visuospatial functions, attention, sequencing, picture priming, recognition of familiar songs, and discrimination of rhythm, meter, transposition, and major from minor keys, but pursuit rotor learning remained intact. By the third year of testing, ML also exhibited severe limb apraxia, but showed only subtle losses in the quality of her piano playing of familiar songs. She also showed immediate and accurate transfer of her playing skill from the piano to the xylophone. Preserved musical performance in AD seems to depend on circuits involving the basal ganglia, cerebellum and motor areas of the thalamus and cerebral cortex that remain relatively intact until the late stages of the disemse, but there is no evidence that the right hemisphere plays a special role in this phenomenon.     

A case report of dementia with cluttering-like speech disorder and apraxia of gait]

A 57-year-old woman presented with a slowly progressive gait disturbance in 1992 (53 years of age). Over the next year, she gradually began to talk less, but her speech itself became more rapid than before. He speech was frequently too fast even for family members to understand. In 1997, she was admitted to our hospital. On admission, the patient was disoriented but able to follow simple verbal commands, to name things, and to write simple words. Neither apraxia, aphasia, hemispatial neglect, nor a corpus callosum disconnection syndrome was observed. There was no muscle weakness or atrophy. She showed a positive Babinski sign with mild spasticity in the legs and Gegenhalten, but no rigidity. Her speech was monotonous and abnormally fast (cluttering-like speech). Her speech became faster and faster toward the end of sentences, skipping several syllables or even words. She was unable to speak slowly and clearly, even when efforts were made to pace her speech to the speed set by the examiner. She was able to stand only with a wide base of support and body flexion. When standing, she was unable to place one foot directly beside the other; as she tried to have one foot near the other, the former repelled the latter. She had great difficulties in taking her first step forward, and showed rapid freezing of gait even when she managed to succeed in starting. She was able to imitate walking or bicycling with her legs unloaded, indicating that her gait disturbance was a kind of apraxia of gait. Her intelligence was somehow difficult to assess because of her peculiar speech disturbance. However, her family members had noticed her memory disturbance and personality change (offensiveness) since 3 to 4 years before the admission. Moreover, she was defective not only on Hasegawa Damentia Scale-Revised but also on Raven's Colored Progressive Matrices which estimates non-verbal intelligence. It was also noted that she was inattentive and lazy in thinking on questionnaires. Thus we considered that she was at least mildly demented and the type of dementia was of frontal pathology. Laboratory data were all normal except for the head MRI, which demonstrated prominent and thinness of the corpus callosum from the anterior part of the body to splenium without any other brain lesions that could cause the thinness secondarily. Our case resembles two cases reported by Sunohara et al in 1985, together comprising a unique clinical feature. Although Sunohara et al did not refer to the thinness of the corpus callosum in their cases, the clinical profiles in our case and theirs raise the possibility that they form a new disease entity. A further study in a large number of similar cases, including autopsies will provide a conclusion.     

Musical Skill in Dementia: A Violinist Presumed to Have Alzheimer’s Disease Learns to Play a New Song

Previous studies have described patients with possible or probable Alzheimer’s disease (AD) who continued to play familiar songs skillfully, despite their dementias. There are no reports about patients with dementia who successfully learned to play new songs, and two papers describe failures of patients with AD to learn to play a new song although they continued to play familiar songs competently. In the present paper we describe a moderately demented patient (SL) with probable AD who learned to play a song (Cossackaya!) on the violin that was published after the apparent onset of his dementia. He showed modest retention of the song at delays of 0 and 10 minutes. This contrasts with his profound disturbance in both recall and recognition on other anterograde memory tests (word lists, stories, figures, environmental sounds, sounds of musical instruments), and marked impairment on measures of remote memory (famous faces, autobiographical memory). SL showed milder deficits in confrontation naming, verbal fluency and attention, but no dyspraxia or aphasic comprehension deficits. Except for the Block Design test, his visuospatial skills were intact. SL’s learning of the new song in the absence of any evidence of episodic memory is reminiscent of patients with temporal lobe amnesia who show better memory for song melody than for lyrics or verse, although his retention was not as good.     

Musical skill in dementia: a violinist presumed to have Alzheimer's disease learns to play a new song

Previous studies have described patients with possible or probable Alzheimer's disease (AD) who continued to play familiar songs skillfully, despite their dementias. There are no reports about patients with dementia who successfully learned to play new songs, and two papers describe failures of patients with AD to learn to play a new song although they continued to play familiar songs competently. In the present paper we describe a moderately demented patient (SL) with probable AD who learned to play a song (Cossackaya!) on the violin that was published after the apparent onset of his dementia. He showed modest retention of the song at delays of 0 and 10 minutes. This contrasts with his profound disturbance in both recall and recognition on other anterograde memory tests (word lists, stories, figures, environmental sounds, sounds of musical instruments), and marked impairment on measures of remote memory (famous faces, autobiographical memory). SL showed milder deficits in confrontation naming, verbal fluency and attention, but no dyspraxia or aphasic comprehension deficits. Except for the Block Design test, his visuospatial skills were intact. SL's learning of the new song in the absence of any evidence of episodic memory is reminiscent of patients with temporal lobe amnesia who show better memory for song melody than for lyrics or verse, although his retention was not as good.     

A case of left dorsomedial thalamic infarction with unilateral schizophrenia-like auditory hallucinations]

We report a case of a right-handed, 73-year-old woman with auditory hallucinations lateralized to the right ear. A brain MRI revealed a small infarction in the left dorsomedial nucleus (DM) of the thalamus. The patient did not have either psychiatric or neurological prior history, and had otherwise been treated for ischemic heart disease, hypertension, and hyperlipidemia for 10 years. Two months prior to admission, she had become forgetful, and had lost her wallet several times. She concurrently began to experience auditory hallucinations in which she heard the voices of her acquaintances, or "the gods". She frequently monologized and wandered about outside following the contents of the hallucinations. Therefore, she was admitted to our institution. On admission, no apparent abnormalities were revealed by physical examinations or blood analyses. She was alert and had no aphasic symptoms. Except for memory disturbances, no neurological symptoms, including no hearing loss, were found. A brain MRI showed a small localized infarction in the left DM, but EEG findings were normal. The patient had prominent anterograde memory deficits: she hardly remembered what she had done the very same day, or the names of the doctor and hospital. She also demonstrated a retrograde amnesia of the past decade or two: she showed difficulty recalling either personal history or social events that occurred during this era. Wechsler Adult Intelligence Scale-Revised (WAIS-R) revealed a total IQ of 75 (verbal IQ 77; performance IQ 77). The verbal hallucinations continued with frequent occurrence even after admission. They included voices telling her about misfortunes, such as death or sickness, of her relatives. These turned into threats and commands, such as "I'm gonna kill ya. I attack you from behind. You, do not eat!" In addition, she occasionally experienced "third person auditory hallucination", in which several men were discussing the plan to kill her. As is characteristic of this type of case, the hallucinations always appeared in only her right ear. They did not occur in the other modalities (e.g. as a visual one). She was convinced that the hallucinations were real and looked frightened while they were happening. Whereas the anterograde amnesia continued for 6 months after admission, the retrograde amnesia gradually improved within 2 or 3 months after admission, although a partial amnesia on the past decade eventually turned out to persistent. On the other hand, the hallucinations did not ameliorate satisfactorily with risperidone (3-6 mg/day), but on augmentation with olanzapine (5-20 mg/day), they lessened gradually and almost disappeared within 6 months. She also slowly developed symptoms similar to those of frontal lobe syndrome, i.e., aspontaneity and apathy. In conclusion, our case indicates the importance of DM on memory function. It is noteworthy that schizophrenia-like hallucinations developed in the case. Localized neuronal deficits evoked by infarction in the left DM probably caused the schizophrenia-like hallucinations; the lateralization phenomenon further indicates the involvement of specific neuronal mechanisms in the mediation of the hallucinations. According to the knowledge of the functional anatomy of the DM and the lateralization phenomenon of auditory hallucinations, it is possible that the neuronal loop, comprised of the prefrontal cortex and thalamus, designated as "basal ganglia-thalamocortical circuits", in addition to the left temporal cortex, plays an important role in the development of the hallucinations in this case. This possibility might also shed light on the neurological basis of schizophrenia.     

An 80-year-old woman with parkinsonism and progressive dementia]

We report an 80-year-old Japanese woman who presented levodopa-responsible parkinsonism followed by progressive dementia. She was well until her 61 years of age (in 1978) when she noted onset of resting tremor in her right hand followed by tremor in her right leg. She was treated with levodopa and trihexyphenidyl with good response, however, later on, she suffered from gait disturbance. In 1985, she had an episode of cardio-pulmonary arrest from which she was resuscitated, however, she started to show hypermetamorphosis, memory defect, and aggressive behaviors. She also developed motor fluctuations and dyskinesias from levodopa. She was admitted to our service in 1986; she showed rather typical parkinsonism and mild dementia. She received left Vim thalamotomy in the same year. Her dyskinesias improved, however, her gait disturbance became progressively worse. In 1995, she was admitted to our service again; she showed marked dementia and advanced parkinsonism; she was unable to walk unsupported. She became bedridden in 1996 and gastrostomy was placed. She was transferred to Zushi Aoki Hospital. Her dementia became progressively worse, and she was in the akinetic and mute state. She expired on April 22, 1998. She was discussed in a neurological CPC. The chief discussant arrived at a conclusion that the patient had Parkinson's disease with complication by Alzheimer's disease in her later clinical course. The diagnoses of participants were divided among Parkinson's disease with dementia, Parkinson's disease and Alzheimer's disease, and diffuse Lewy body disease. Postmortem examination revealed marked neuronal loss in the substantia nigra and the locus coeruleus. Lewy bodies were found in the substantia nigra. In addition, rather many Lewy bodies of cortical type were seen in the cingulate gylus, inferior temporal gylus, and in the amygdaloid nucleus. These Lewy bodies were positive for alpha-synuclein. Also, tau-positive intra-neuronal tangles were seen in the hippocampus and in the substantia nigra. The Meynert nucleus showed marked neuronal loss. Pathologic findings were consistent with the diagnosis of diffuse Lewy body disease.     

Preservation of absolute pitch after right amygdalohippocampectomy for a pianist with TLE

Absolute pitch (AP) ability is a rare musical phenomenon. In the literature, it has been suggested that the relative specialization for pitch processing is in the right temporal lobe in the non-AP population. Since the anatomic basis for absolute pitch is not fully understood and cases of temporal lobe epilepsy of AP possessors are extremely rare, applicability of resection as a treatment of epilepsy in this particular area should be evaluated with caution. In the present study, we examined an AP possessor who suffered from medically refractory temporal lobe epilepsy and underwent right selective amygdalohippocampectomy (SAH). The SAH procedure clearly avoided disturbing important structures for AP, inasmuch as postsurgically she preserved her AP ability and was seizure-free. She did well post-operatively in the test of pure sine wave tones with short reaction time, which could be identified as “true” absolute pitch.     

Recurrent unipolar depression with 48-hour cycle and the state--dependent abnormal involuntary movements following right thalamic hemorrhage]

A 70-year-old woman was admitted because of depression and abnormal involuntary movements of her left extremities. Six months before the admission, she developed left hemiparesis caused by right thalamic hemorrhage. On neurological examination, she had mild motor and sensory hemiparesis on the left side. She showed recurrent depression each other days with a 48-hour cycle, and hemichorea-hemiballism appeared in her left upper and lower extremities exclusively on the days with depressive phase. Her depressive symptoms were characterized by psychomotor retardation but not by feeling of suicide or guilty. She was then diagnosed as having a rapid cycler following the right thalamic hemorrhage. To our knowledge, it is extremely rare to see the state-dependent involuntary movements in patients with rapid cycler. The coexistence of post-stroke depression and hemichorea-hemiballism in the present patient may suggest that both disorders appear on the basis of the common pathophysiological mechanism such as dysfunction on frontal cortico-basal ganglia-thalamo-cortical loops.     

Traumatic intraventricular hemorrhage with a good prognosis

We report a 10-year-old girl with an isolated traumatic intraventricular hemorrhage following a traffic accident, who had a good prognosis. Her neurological examination upon arrival was normal and she had no complaint other than headache and vomiting. Computed tomography on admission showed a hemorrhage in the lateral and fourth ventricles. She had a Glasgow Coma Score of 15, and she was thus given only antiepileptic drugs for prophylaxis and followed. Computed tomography that was repeated 5 days after admission showed no blood and all ventricles were of normal size. There was no vascular pathology on magnetic resonance imaging and magnetic resonance angiography. The patient remains well 5 months after her accident. Intraventricular hemorrhage does not always have a poor prognosis.     

A 68-year-old woman with dementia and parkinsonism]

We report a 68-year-old woman who developed progressive dementia and parkinsonism. She was well until 1990 when she was 58 years of age. She started to show memory loss. Four years later, she developed difficulty in dressing and behavioral problems such as eating rice with her hands, going out of her house without purposes, and difficulty in finding the rest room in her house. She was admitted to the neurology service of Hatsuishi Hospital on January 19, 1996, when she was 64 years of the age. On admission, she was alert but markedly demented. The score of Hansegawa Dementia Scale was 0/30. She was unable to make any coherent conversation. She appeared to have dressing apraxia but did not appear to have aphasia. Cranial nerves were intact. She walked in small steps with stooped posture. She did not have motor weakness but she showed plastic rigidity in all four limbs. No tremor or ataxia was noted. Deep tendon reflexes were within normal limits but the plantar response was extensor bilaterally. She continued to deteriorate after admission. In May of 1998, she started to fall. In June of 1998, she had a generalized convulsion. In January of 1999, she became unable to take foods orally and a gastrostomy was placed. She expired on May 29, 1990. She was discussed in a neurological CPC and the chief discussant arrived at the conclusion that the patient had Alzheimer's disease. The question was whether her parkinsonism was a part of her Alzheimer's disease or she had an additional disease to explain her parkinsonism. Post-mortem examination revealed moderate to marked atrophy of the frontal and the temporal lobes as well as in the limbic areas with dilatation of the lateral ventricles. Marked neuronal loss was noted in the CA 1 to the subiculum region with gliosis. Neurofibrillary tangles were seen in the remaining neurons. Neuropil threads were seen by Gallyas-Braak staining. Similar changes were seen in the parahippocampal gyrus and in the entorhinal cortex. Senile plaques were seen in the insular cortex and in other cortical areas. Cortical type Lewy bodies were seen in the cingulate cortex. The Meynert nucleus showed marked neuronal loss and gliosis. The substantia nigra and the locus coeruleus showed moderate loss of pigmented neurons. Lewy bodies were seen in these regions. The dorsal motor nucleus of the vagal nerve was retained, however, one Lewy body was observed. Pathologic diagnosis was Alzheimer's disease plus Parkinson's disease. It is an interesting question whether or not her parkinsonism was due to nigral lesion or frontal lesions. It is known that parkinsonism may complicate in advanced Alzheimer's disease not necessarily due to nigral lesion. On the other hand, in incidental Lewy body disease, the substantia nigra shows mild Parkinson's disease-like change without clinical parkinsonism. This patient appeared to have been a true complication of Alzheimer's disease and Parkinson's disease.     

A case of primary progressive multiple sclerosis with onset of memory impairment]

We report a 52-year-old woman with primary progressive multiple sclerosis (PPMS) presenting with chronic progressive memory impairment. From a couple of years prior to admission, she had developed impairment of her short-term memory. For example, she forgot her nephew's name, and spoke the same phrases again and again. She also sometimes forgot to turn off her gas stove and forgot things she bought in shops. Moreover, her mental activity gradually decreased and she became apathetic. However, she did not note her memory impairment, and had no hallucinations. She was admitted to our hospital on 20 May, 2003 because donepezil had been ineffective for treating her memory impairment. Neurologically, she showed bilateral horizontal gaze nystagmus, mild limb ataxia on the left and mildly ataxic gait. Neuropsychological examinations showed mildly impaired cognitive function, e.g., MMSE 25/30, WAIS-R full IQ 69 and especially in verbal short memory, which may represent temporal lobe dysfunction. Moreover, Benton's visual memory test revealed marked visual short-term memory impairment, while impaired performance on a Kana picking up test suggested mild to moderate attention impairment, which could have represented frontal lobe dysfunction. Brain MRI showed multiple T2-high plaque lesions close to the bilateral lateral ventricles, and bilateral optic nerve lesions enhanced by gadolinium. Also, spinal cord MRI showed a gadolinium enhanced lesion at Th5 on the left. Cerebral spinal fluid (CSF) examination showed normal cell count and protein level, and undetectable oligoclonal bands (OCB), but an elevated IgG index (1.1, normal < 0.85). Visual evoked potentials (VEPs) showed prolonged P100 latency bilaterally, indicating subclinical optic nerve lesions. She was thus diagnosed as having PPMS according to McDonald's diagnostic criteria for MS. 99mTc Single photon emission computed tomography (SPECT) showed a decreased cerebral blood flow (CBF) in the bilateral frontal and temporal lobes, which was consistent with her clinical features. PPMS patients generally present with chronic progressive spastic paraparesis and/or cerebellar ataxia. Cognitive impairments observed in PPMS are generally thought to be due to white matter lesions, i.e., subcortical dementia. However, some recent reports have shown MS patients with short-term memory impairment (antegrade amnesia) similar to cortical dementias such as Alzheimer's disease (AD). In such MS cases, visual short-term memory impairment seems characteristic of their cognitive impairment compared to AD cases. As well, the present case showed visual memory impairment as evaluated by Benton's memory test. Parietal and frontal lobes are reported to be important for verbal and visual working memory, respectively. Thus, in the present case, decreased CBF in the frontal and temporal lobes, which could have been due to a disconnection between cortices and subcortices caused by the white matter lesions, is consistent with the type of her cognitive dysfunction, i.e., notable visual memory impairment. PPMS may thus be an important disease as a differential diagnosis for chronic progressive dementia. Further neuropsychological and functional imaging studies will be necessary to achieve a better understanding of the mechanisms of cognitive impairment in PPMS.     

Perception of musical timbre in congenital amusia: categorization, discrimination and short-term memory

Congenital amusia is a neurodevelopmental disorder that is characterized primarily by difficulties in the pitch domain. The aim of the present study was to investigate the perception of musical timbre in a group of individuals with congenital amusia by probing discrimination and short-term memory for real-world timbral stimuli as well as examining the ability of these individuals to sort instrumental tones according to their timbral similarity. Thirteen amusic individuals were matched with thirteen non-amusic controls on a range of background variables. The discrimination task included stimuli of two different durations and pairings of instrumental tones that reflected varying distances in a perceptual timbre space. Performance in the discrimination task was at ceiling for both groups. In contrast, amusic individuals scored lower than controls on the short-term timbral memory task. Amusic individuals also performed worse than controls on the sorting task, suggesting differences in the higher-order representation of musical timbre. These findings add to the emerging picture of amusia as a disorder that has consequences for the perception and memory of musical timbre, as well as pitch.     

An 84-year-old woman with progressive mental deterioration and abnormal behavior]

We report an 84-year-old woman with progressive mental deterioration. She was well until January 1994, when she was 80 years of the age. At that time she developed a delusional ideation, in that she stated that she would be killed by her fellow members of the society for elderly, in which she was belonging. At times, she closed the shutter of her house saying that a stranger was wandering outside of her house. In 1995, she could not identify the face of her son's wife. When she went out for shopping, she lost her way to the home. She prowled about in and out of her home. In 1996, she had to be admitted to a nursing home, where quarrelled with other patients and behaved violently. She was admitted to the neurology service of Hatsuishi Hospital on November 20th, 1997. Family history revealed that her mother was said to be demented. On admission, she was alert and behaved in a good manner. She was disoriented to the time and unable to do serial 7. Her memory was very poor. She did not show aphasia or apraxia. Cranial nerves appeared to be intact. She showed no weakness or muscle atrophy. Gait was normal for her age. Plastic rigidity was noted in four limbs more on the right side. No ataxia was noted. Deep tendon reflexes were exaggerated, however, no Babinski sign was noted. Sensory examination was intact. Her hospital course was characterized by the development of progressive gait disturbance, violent behaviour, and prowling around. On November 30th, 1998, she fell down and suffered from a fracture in the neck of her femur. Although replacement of the femur head was performed, she became unable to walk after this episode. Her mental functions deteriorated further. She developed pneumonia and expired on February 2, 1999. She was discussed in a neurological CPC and the chief discussant arrived at a conclusion that the patient probably had diffuse Lewy body disease, because of the combination of dementia and parkinsonism. Other possibilities discussed in the CPC included Pick's disease, frontotemporal dementia and parkinsonism, and Alzheimer's disease. Post-mortem examination revealed moderate atrophy in the frontal and temporal cortices. Microscopic examination showed atrophy and gliosis in the hippocampus. Many diffuse plaque and neuritic plaques were seen in the frontal cortex by methenamine silver staining. Neurofibrillary tangles were also found. The Meynert nucleus was preserved. The putamen and the substantia nigra were also intact. Pathologic diagnosis was consistent with Alzheimer's disease.     

Do not overlook acute isoniazid poisoning in children with status epilepticus

A previously healthy 2-year-old girl was admitted with generalized convulsive status epilepticus. She was in a stupor and could respond only to painful stimuli. She also had severe metabolic acidosis. Although initial liver function tests were normal, they were found to be moderately high on the fifth day of admission; however, they dropped to their normal ranges on the twelfth day of admission. Initially, the patient was diagnosed as having idiopathic status epilepticus, and classic anticonvulsant agents, including diazepam, phenytoin, and then phenobarbital, were given. However, her seizures did not subside, and diazepam infusion was initiated. After initiation of diazepam infusion, the seizures were completely controlled. On the fourth day of admission, her parents said that she had accidentally received 20 tablets (a total dose of 2000 mg) of isoniazid just before admission to our hospital. Later, we injected 200 mg of pyridoxine intravenously. During follow-up, her general condition improved, and anticonvulsant agents were discontinued because an electroencephalogram was found to be norma. She was discharged from the hospital on the twelfth day of admission. At the fourth month of follow-up, she was seizure free. Because of this case, we would like to re-emphasize that acute isoniazid poisoning should also be considered in a child with unexplained status epilepticus.