Longitudinal evaluation of early Alzheimer's disease using brain perfusion SPECT.

UNLABELLED: The aim of this SPECT study was to determine the initial abnormality and longitudinal changes in regional cerebral blood flow (rCBF) in early Alzheimer's disease (AD) using statistical parametric mapping (SPM). METHODS: rCBF was noninvasively measured using (99m)Tc-ethyl cysteinate dimer SPECT in 32 patients complaining of mild cognitive impairment, with a Mini-Mental State Examination score more than 24 at the initial study, and 45 age-matched healthy volunteers. All patients satisfied the diagnostic criteria of AD during the follow-up period of at least 2 y. Follow-up SPECT studies were performed on the patients at a mean interval of 15 mo. We used the raw data (absolute rCBF parametric maps) and the adjusted rCBF images of relative flow distribution (normalization of global cerebral blood flow [CBF] for each subject to 50 mL/100 g/min with proportional scaling) to compare these groups with SPM. RESULTS: In the baseline study, the adjusted rCBF was significantly and bilaterally decreased in the posterior cingulate gyri and precunei of patients compared with healthy volunteers. In the follow-up study, selected reduction of the adjusted rCBF was observed in the left hippocampus and parahippocampal gyrus. These areas showed the most prominent reduction in absolute rCBF on each occasion. Moreover, further decline of the absolute rCBF was longitudinally observed in extensive areas of the cerebral association cortex. CONCLUSION: SPM analysis showed the characteristic early-AD rCBF pattern of selective decrease and longitudinal decline, which may be overlooked by a conventional region-of-interest technique with observer a priori choice and hypothesis. This alteration in rCBF may closely relate to the pathophysiologic process of this disease.

Chiral-symmetry realization for even- and odd-parity baryon resonances

Baryon resonances with even and odd parity are collectively investigated from the viewpoint of chiral symmetry (ChS). We propose a quartet scheme where Delta's and N(*)'s with even and odd parity form a chiral multiplet. This scheme gives parameter-free constraints on the baryon masses in the quartet, which are consistent with observed masses with spin 1 / 2,3 / 2,5 / 2. The scheme also gives selection rules in the one-pion decay: The absence of the parity nonchanging decay N(1720)-->piDelta(1232) is a typical example which should be confirmed experimentally to unravel the role of ChS in baryon resonances.

Cyclic GMP inhibits and shifts the activation curve of the delayed-rectifier (I[K1]) of type I mammalian vestibular hair cells.

NITRIC oxide (NO) plays a role in the modulation of the predominant potassium current of type I vestibular hair cells, a low-voltage activated current called I(K1). Since many effects of NO are mediated via cGMP, patch-clamp recordings were made to evaluate the effects of cGMP on I(K1). In whole-cell recordings 1 mM cGMP shifted Vhalf of I(K1) by 15.0 +/- 2.4 mV (n = 6) to more positive. In cell-attached 'multichannel' recordings 1 mM 8-bromo-cGMP caused a reversible shift of Vhalf by 13.8 +/- 2.6 mV (n = 12) and in single channel recordings in the cell-attached configuration the open probability was reduced at -60 mV from 0.39 +/- 0.14 to 0.08 +/- 0.01. cGMP had no effect on excised inside-out patches, indicating that an intact cytosolic milieu with functioning phosphorylation cascades is necessary. cGMP seems to be an important second messenger which reduces the potassium conductance of vestibular hair cells.

Cowden's disease diagnosed through mucocutaneous lesions and gastrointestinal polyposis with recurrent hematochezia, unrevealed by initial diagnosis.

A 51-year-old man was admitted to our hospital because of hematochezia and painful keratotic plaques involving both hands. He had gastrointestinal polyposis, and a history of liver hemangiomas and thyroid tumor. Numerous papules on the face and papillomatosis on the oral mucosa were present. A diagnosis was made as a typical case of Cowden's disease according to the criteria proposed by Salem and Steck (J Am Acad Dermatol 8: 686, 1983). The patient was not correctly diagnosed initially in spite of typical manifestations of Cowden's disease, mainly due to his concomitant manifestations which occurred chronologically.

Primary adenosquamous carcinoma of the liver which produces granulocyte-colony-stimulating factor and parathyroid hormone related protein: association with leukocytosis and hypercalcemia.

A 55-year-old man was admitted to our hospital with fever and vomiting. Abdominal computed tomography (CT) revealed multiple low density masses in the liver. A diagnosis of primary adenosquamous carcinoma of the liver was confirmed by histological examination of a necropsy specimen. The present case showed leukocytosis and hypercalcemia with high levels of serum granulocyte-colony-stimulating factor (G-CSF) and parathyroid hormone related protein (PTHrP). Recent studies have shown that G-CSF and PTHrP are responsible for the paraneoplastic syndromes with leukocytosis and hypercalcemia. The tumor cells demonstrated positive cytoplasmic immunohistochemistry staining with anti-G-CSF and anti-PTHrP antibodies. This result suggested that the tumor produced G-CSF and PTHrP.

Perilymph fistula--45 case analysis.

PURPOSE OF THE STUDY: Though perilymph fistula (PLF) is not a rare disease, preoperative diagnosis still remains to be established. Some new diagnostic methods are challenging, but there is still no established diagnostic method except exploratory tympanotomy that verifies the occurrence of leakage. Early diagnosis of PLF is fully depending on history taking and some clinical examinations. To know the clinical features of PLF is one of the greatest helps to make both earlier and accurate diagnosis. In spite of some innovations in clinical examinations classic diagnostic procedure is thought to be still reliable. PROCEDURES: We investigated the clinical symptoms, basic tests results and therapeutic results in patients with PLF. RESULTS: From 1983 to 1998 PLF was identified in 44 patients (45 ears) with exploratory tympanotomies in our hospital. With respect to clinical history the predisposing factors such as blowing the nose, lifting heavy goods, and landing in an airplane were found in almost half of the patients, while the rest of them had no clear inducing factors. Their major symptoms included hearing loss (93%), vertigo and dizziness (91%), tinnitus (76%), and aural fullness (31%). The patients who have a clear predisposing factor tended to make diagnosis easily; on the other hand the rest of the patients who do not have clear etiology had some diagnostic difficulty. Subjective positive fistula signs were observed in 71% of patients. Vestibular symptoms improved in 80% of patients after closure of PLF. CONCLUSIONS: These results suggest that the variety of clinical manifestation make diagnosis more difficult. At the moment meticulous clinical history taking and close follow-up applying repeating fistula tests are the most important for not only earlier but also accurate diagnosis.

[Longitudinal evaluation of early dementia of Alzheimer type using brain perfusion SPECT].

Regional cerebral blood flow (rCBF) measurements using a Patlak plot method of 99mTc-ECD were performed in early dementia of Alzheimer type (DAT) with both HDS-R and MMSE of over 20 to investigate initial abnormality and longitudinal changes of rCBF. A fusion technique of MRI and SPECT images was developed for MRI-guided analysis of regions of interest in hippocampal areas and statistical parametric mapping (SPM) was used for automated and objective approach to analysis of SPECT image data. Seventeen patients with clinically diagnosed early DAT and age-matched 32 normal control subjects were studied. At the first SPECT studies, the mean cerebral blood flow (mCBF) of 38.6 +/- 4.7 ml/100 g/min (mean +/- SD) for early DAT did not show significant reduction as compared with the normal control value of 42.0 +/- 3.8, whereas the rCBF values in the bilateral hippocampi (right; 26.8 +/- 4.7, left; 26.7 +/- 5.2) showed significant reduction (p < 0.05) as compared with the normal control values (right; 38.3 +/- 4.2, left; 38.4 +/- 3.8). The SPM analysis (voxel height; p < 0.001, Bonferroni correction; p < 0.05) of the first SPECT images revealed significant selective decrease of relative rCBF in the bilateral posterior cingulate gyri. At the second SPECT studies after 1.4 year on the average from the first studies, mCBF for early DAT showed a slight decrease by 1.7 +/- 3.8 ml/100 g/min/year. Bilateral hippocampi showed a greater decrease with slight left-side dominance by 3.8 +/- 3.3 on the right and 4.4 +/- 3.2 on the left side. The SPM analysis demonstrated significant decrease of relative rCBF in the basal fore-brain area, the left hippocampus, the left amygdala, and the left parahippocampal area. These results suggest that the MRI-guided ROI analysis of rCBF values in the hippocampus and the SPM analysis of SPECT images are quite useful for early diagnosis and follow-up of DAT.

The oxygen isotopic composition of the Sun inferred from captured solar wind.

All planetary materials sampled thus far vary in their relative abundance of the major isotope of oxygen, (16)O, such that it has not been possible to define a primordial solar system composition. We measured the oxygen isotopic composition of solar wind captured and returned to Earth by NASA's Genesis mission. Our results demonstrate that the Sun is highly enriched in (16)O relative to the Earth, Moon, Mars, and bulk meteorites. Because the solar photosphere preserves the average isotopic composition of the solar system for elements heavier than lithium, we conclude that essentially all rocky materials in the inner solar system were enriched in (17)O and (18)O, relative to (16)O, by ~7%, probably via non-mass-dependent chemistry before accretion of the first planetesimals.

Does hearing preservation surgery for acoustic neuromas affect tinnitus?

Questionnaires returned from 202 patients who had undergone acoustic neuroma resection were analyzed with special reference to the effect on tinnitus of surgery that attempted to preserve hearing versus surgery that did not. We also examined the relationship between postoperative hearing and tinnitus following surgery to preserve hearing (103 patients, HP group). The incidence of preoperative tinnitus in the HP group was 78.6%; postoperatively it increased to 89.3% (p = 0.0367). The remaining 99 patients (non-HP) underwent labyrinthectomy during tumor resection. In this group tinnitus occurred in 72.7% preoperatively and in 67.3% postoperatively (p = 0.4097). Our findings show that when tinnitus was absent preoperatively, 85% of the HP group developed it postoperatively compared to 31% of patients in the non-HP group. The outcome of surgery to preserve hearing had no bearing on the loudness or annoyance of postoperative tinnitus. Although postoperative tinnitus is tolerable to most patients, they must be informed prior to surgery about its possible occurrence or worsening, especially if the goal is to preserve hearing.

Analysis of the prognosis and the recovery process of profound facial nerve paralysis secondary to acoustic neuroma resection.

The prognosis and the recovery process of facial nerve paralysis were reviewed in 74 patients who, despite preservation of nerve continuity, showed no facial movement after acoustic neuroma resection. In 50 or 67.6% of patients, facial movement recovered sufficiently so as not to require any reanimation procedures. However, no apparent sign of remission was observed for 7-49 months in the other 24 patients (32.4%), and hypoglossal-facial nerve anastomosis was performed in 20 of these patients. When remission was seen, the first sign of muscle movement appeared most frequently after 3-4 months but, in a small number of patients, it was also seen within 1.5 months or after 5-10 months. Based upon these results, the timing of reanimation procedures for facial nerve paralysis following acoustic neuroma resection is discussed.

The evaluation of facial palsy by amount of feature point movements at facial expressions.

At present, in the medical field 40-point method and facial nerve grading system are generally used for evaluation of facial palsy. However, these methods have limitation in the precise evaluation, because of subjectivity in diagnosis. Purpose of This work is to propose quantitative evaluation of facial palsy based on the amount of movements of the feature point on the face. Facial nerve symptoms generally appear in either side of the face. In facial expression movement, the motion in the palsy side becomes smaller than that of the healthy side. We defined some indices of palsy grade obtained by the observation of the facial motion. Those indices showed the asymmetry of the facial motion quantitatively. We confirmed that our proposed method is valid for estimation of facial palsy from comparison with 40 points method.

Hypoglossal-facial nerve anastomosis. Clinical observation.

We have reviewed 35 cases of hypoglossal-facial nerve anastomosis performed during the past 15 years for irreversible peripheral facial paralysis caused by surgery for acoustic neuroma. Of 27 patients who were followed more than 1 year after anastomosis, recovery of serviceable facial function was obtained in 25 (92.6%). Neural deficits secondary to transection of the hypoglossal-facial nerve were minimal or acceptable in most cases. The overall results were better in patients who underwent this procedure within 3 months after surgery for acoustic neuroma as compared with those who did so after 1 year or more. The 2 patients who underwent intracranial facial nerve reconstruction during surgery for acoustic neuroma and showed poor facial recovery have presented a challenge to our strategy in the treatment of such patients.

Steroid-responsive sensorineural hearing loss associated with aortitis syndrome.

Five cases of sensorineural hearing loss associated with aortitis syndrome are presented, and their clinical features are discussed in detail. All patients were middle-aged females. Pure-tone audiometry revealed a high-tone gradual-loss type of configuration, and the recruitment phenomenon was proved to be positive in most cases. The degree of hearing loss correlated well with the erythrocyte sedimentation rate. However, the most remarkable clinical feature was that the hearing loss showed steroid responsiveness in all cases. Based on these clinical features, it was suggested that the steroid-responsive sensorineural hearing loss associated with this syndrome might not be an incidental accompanying symptom but rather one of the local manifestations of the disease arising from similar mechanisms as the systemic inflammatory process. Associated conductive disturbance is also discussed.

Abnormal regional cerebral blood flow in childhood autism.

Neuroimaging studies of autism have shown abnormalities in the limbic system and cerebellar circuits and additional sites. These findings are not, however, specific or consistent enough to build up a coherent theory of the origin and nature of the brain abnormality in autistic patients. Twenty-three children with infantile autism and 26 non-autistic controls matched for IQ and age were examined using brain-perfusion single photon emission computed tomography with technetium-99m ethyl cysteinate dimer. In autistic subjects, we assessed the relationship between regional cerebral blood flow (rCBF) and symptom profiles. Images were anatomically normalized, and voxel-by-voxel analyses were performed. Decreases in rCBF in autistic patients compared with the control group were identified in the bilateral insula, superior temporal gyri and left prefrontal cortices. Analysis of the correlations between syndrome scores and rCBF revealed that each syndrome was associated with a specific pattern of perfusion in the limbic system and the medial prefrontal cortex. The results confirmed the associations of (i) impairments in communication and social interaction that are thought to be related to deficits in the theory of mind (ToM) with altered perfusion in the medial prefrontal cortex and anterior cingulate gyrus, and (ii) the obsessive desire for sameness with altered perfusion in the right medial temporal lobe. The perfusion abnormalities seem to be related to the cognitive dysfunction observed in autism, such as deficits in ToM, abnormal responses to sensory stimuli, and the obsessive desire for sameness. The perfusion patterns suggest possible locations of abnormalities of brain function underlying abnormal behaviour patterns in autistic individuals.

Electromyographic analysis of profound facial nerve paralysis following acoustic neuroma resection.

The aim of our retrospective study was to determine whether electromyographic findings (motor unit action potentials, MUAPs) can be used in long-term prognosis for profound facial nerve paralysis in patients whose nerve continuity is preserved during surgery for acoustic neuroma. The orbicularis oris, frontal, and orbicularis oculi muscles were examined for the occurrence of MUAPs in 48 such patients. In 30 patients who recovered from complete paralysis within 10 months after surgery, MUAPs in the first two muscles tended to precede the first sign of facial movement. MUAPs appeared in the orbicularis muscle in 80% of these patients at 1 month and in all at 5 months. In the frontal and orbicularis oculi muscles, MUAPs occurred in only 0-20% of these patients in the first month; within 3-5 months the number increased rapidly, and MUAPs were present in 95% of these patients at 10 months. In the remaining 18 patients with long-term complete paralysis (at least 1 year), MUAPs appeared solely in the orbicularis oris muscle: in 20% of these patients in the first month after surgery. While this number slowly rose, there was no period of rapid increase later. We conclude that the occurrence of MUAPs in the orbicularis oris and frontal muscles within 3 months of surgery indicates a good prognosis for reversal of facial nerve paralysis.

Long-term prognosis of profound facial nerve paralysis secondary to acoustic neuroma resection.

The long-term prognosis of profound facial nerve paralysis was reviewed in 107 patients who, despite preserved nerve continuity, showed no facial movement after acoustic neuroma resection. Spontaneous recovery occurred in 77 patients. However, there was no apparent recovery in 30 patients. Twenty-two of these patients underwent hypoglossal-facial nerve anastomosis 7-33 months after tumor resection. When spontaneous recovery occurred, the first sign of remission was observed between 3 and 4 months after surgery in nearly half of the patients. Such a sign did not appear after 12 months. The recovery of facial movement deteriorated depending on how long remission onset was delayed. However, the quality of facial movement in patients with such delayed remission was still identical or better than that in those after hypoglossal-facial nerve anastomosis. These results showed that hypoglossal-facial nerve anastomosis should be performed approximately 1 year after tumor resection if no sign of remission has been observed by then.

Hypoglossal-facial nerve anastomosis after acoustic neuroma resection: influence of the time anastomosis on recovery of facial movement.

Facial movement following hypoglossal-facial nerve anastomosis was investigated in 29 acoustic neuroma patients. The amount of facial movement was assessed using both the grading system of House and Brackmann and the revised grading scale of Yanagihara. The data were analyzed to determine the influence of the time elapsed between tumor resection and anastomosis upon recovery of facial movement. A slightly larger number of patients with delayed anastomosis (7-23 months) showed minimally poorer results than those with early anastomosis (within 3 months). However, these differences were not statistically significant. Moreover, there was no apparent relationship between the duration of facial nerve paralysis and the recovery of facial movement within either of these two groups. These results showed that hypoglossal-facial nerve anastomosis can be delayed up to 2 years following tumor resection with only minimal effect on the recovery of facial movement.

Preservation of facial nerve function in acoustic neuroma surgery by the extended middle cranial fossa approach.

The anatomical preservation rates of the facial nerve and postoperative facial nerve function were investigated in cases of initial operation for acoustic neuroma by the middle cranial fossa or extended middle cranial fossa approach. The cases were divided chronologically into three groups according to the date of surgery. The rate of anatomical preservation was 93% in the most recent period, compared to 82.4% for the entire series. This was attributable to higher preservation rates being achieved in cases with medium or large tumors with increased experience. Also regarding postoperative facial nerve function, the number of cases with no paralysis or only partial paralysis increased and the number of cases requiring sacrifice of the facial nerve decreased as experience was accumulated in the series. Even when the facial nerve was preserved anatomically, however, facial-hypoglossal anastomosis was carried out actively if facial nerve function did not recover satisfactorily one year after surgery. For this reason, as many as 33% of the patients underwent anastomosis. This high percentage, however, is attributable to anastomosis ultimately being carried out in 47.7% of the patients in the early period; 22.2% of the patients in the most recent period underwent anastomosis.

Intracranial reconstruction of the facial nerve. Clinical observation.

Nine cases of intracranial facial nerve reconstruction are reviewed in this paper. All patients underwent this procedure for severe injury or disruption of the facial nerve during surgery for acoustic neruroma through the modified extended middle cranial fossa approach (1). Satisfactory recovery of facial function was obtained in 4 patients. Three patients underwent hypoglossal-facial nerve anastomosis 1.3-1.5 years later for no or poor recovery of the facial function. One patient refused any further surgical treatment despite unsatisfactory recovery. The remaining 1 patient, during a telephone interview, stated that facial function had not returned at all 1 year and 5 months postoperatively. Although some degree of associated movement or mass movement was unavoidable, facial movement and mimetic facial expression were better in the patients with satisfactory recovery, as compared with those after hypoglossal-facial nerve anastomosis (2). Fibrin glue, which we used in the latest 3 cases instead of suture, seemed to possibly solve the technical difficulty in placing a suture. Facial function after intracranial reconstruction with fibrin glue was as good or better than that after repair by suturing.