Two cases of fronto-temporal dementia without remarkable lobar atrophy]

Front-temporal dementia (FTD), advocated by Lund and Manchester groups, includes Pick type corresponding to the conventional frontal Pick's disease, motor-neuron type associated with neural symptoms, and frontal lobe degeneration type. In Japan, however, there have been few case reports of the frontal lobe degeneration type. Here we examined clinical characteristics and imaging findings of 2 cases of FTD frontal lobe degeneration type. Neurological examinations were normal. CT and MRI scans revealed no obvious frontal lobar atrophy, while HMPAO-SPECT scans demonstrated remarkable hypoperfusion in anterior hemisphere. Neuropsychological examination revealed frontal symptoms, including personality change, stereotypes, and disinhibition. These symptoms can not be distinguished from the cases of FTD Pick type, because those cases of FTD Pick type have obvious lobar atrophy.     

Basal ganglia, thalamus and neocortical atrophy predicting slowed cognitive processing in multiple sclerosis

Information-processing speed (IPS) slowing is a primary cognitive deficit in multiple sclerosis (MS). Basal ganglia, thalamus and neocortex are thought to have a key role for efficient information-processing, yet the specific relative contribution of these structures for MS-related IPS impairment is poorly understood. To determine if basal ganglia and thalamus atrophy independently contribute to visual and auditory IPS impairment in MS, after controlling for the influence of neocortical volume, we enrolled 86 consecutive MS patients and 25 normal controls undergoing 3T brain MRI and neuropsychological testing. Using Sienax and FIRST software, neocortical and deep gray matter (DGM) volumes were calculated. Neuropsychological testing contributed measures of auditory and visual IPS using the Paced Auditory Serial Addition Test (PASAT) and the Symbol Digit Modalities Test (SDMT), respectively. MS patients exhibited significantly slower IPS relative to controls and showed reduction in neocortex, caudate, putamen, globus pallidus, thalamus and nucleus accumbens volume. SDMT and PASAT were significantly correlated with all DGM regions. These effects were mitigated by controlling for the effects of neocortical volume, but all DGM volumes remained significantly correlated with SDMT, putamen (r = 0.409, p < 0.001) and thalamus (r = 0.362, p < 0.001) having the strongest effects, whereas for PASAT, the correlation was significant for putamen (r = 0.313, p < 0.01) but not for thalamus. We confirm the significant role of thalamus atrophy in MS-related IPS slowing and find that putamen atrophy is also a significant contributor to this disorder. These DGM structures have independent, significant roles, after controlling for the influence of neocortex atrophy.     

Efficacy of hippocampal transection for left temporal lobe epilepsy without hippocampal atrophy

We describe a case of left temporal lobe epilepsy without hippocampal atrophy. A 31-year-old woman presented with typical symptoms of complex partial seizures. Magnetic resonance imaging demonstrated slightly obscure internal structures in the left hippocampus. Scalp electroencephalography revealed interictal epileptiform discharges in the left temporal lobe. A Wada test with propofol determined the language-dominant hemisphere to be the left. Intraoperative electrocorticography revealed active epileptic discharges in the hippocampus and the anterior temporal basal area. The hippocampal epileptic area was treated with multiple transection, which led to the complete cessation of epileptic discharges. After surgery, the Rey Auditory Verbal Learning Test score decreased from 12 to 9. However, it returned to the preoperative level 6months after surgery. We describe this case as a typical example demonstrating the efficacy of hippocampal transection for seizure control and the preservation of verbal memory.     

Video-EEG monitoring in patients with hippocampal atrophy

OBJECTIVES: To analyze the value of ictal EEG recordings in patients with unilateral magnetic resonance imaging (MRI)-identified hippocampal atrophy and concordant interictal epileptiform discharges (IEDs). MATERIAL AND METHODS: The ictal EEG patterns in 84 patients with pharmaco resistant epilepsy undergoing an anterior temporal lobectomy between 1992 and 1995 were reviewed. The concordance between the ictal EEG and MRI and the IEDs was examined. RESULTS: Two-hundred-and-thirty seizures (76.4%) were concordant and 11 seizures (3.7%) were discordant with the atrophic temporal lobe. Sixty seizures (19.9%) were indeterminate in localization. Sixty-three of the 69 patients (91.3%) with confirmatory and 14 of the 15 patients (93%) with non-confirmatory ictal EEG recordings, respectively, experienced an excellent operative outcome (P=0.629, Fisher's exact test). CONCLUSIONS: Patients with unilateral hippocampal atrophy and concordant IEDs are excellent surgical candidates even when video-EEG monitoring shows discordant or non-localizing seizures.     

Progressive hippocampal atrophy in chronic intractable temporal lobe epilepsy

We report on a 28-year-old man with long-standing intractable complex partial and secondary generalized seizures, whose magnetic resonance imaging scans 4 years apart documented progressive decrease in the left hippocampal volume. Left anterior temporal lobectomy with amygdalohippocampectomy rendered the patient seizure free at 12 months' follow-up. The findings demonstrate that patients with uncontrolled temporal lobe seizures may develop progressive atrophy of the hippocampus, in the absence of status epilepticus.     

Basal ganglia alterations and brain atrophy in Huntington's disease depicted by transcranial real time sonography

OBJECTIVES AND METHODS: Transcranial real time sonography (TCS) was applied to 49 patients with Huntington's disease and 39 control subjects to visualise alterations in the echotexture of the basal ganglia. For comparison T1 weighted, T2 weighted, and fast spin echo MRI was performed in 12 patients with Huntington's disease with and in nine patients without alterations of the basal ganglia echotexture as detected by TCS and T1 weighted, T2 weighted, and fast spin echo MRI. Furthermore, the widths of the frontal horns, third ventricle, and the lateral ventricles were depicted in TCS examinations and correlations examined with corresponding CT slices. RESULTS: Eighteen out of 45 (40%) of the patients with Huntington's disease with adequate insonation conditions showed hyperechogenic lesions of at least one basal ganglia region. In 12 patients TCS depicted hyperechogenic lesions of the substantia nigra; in six patients the head of the caudate nucleus was affected. The lentiform nucleus (n=3) and the thalamus (n=0) were less often affected or spared. Hyperechogenic lesions were significantly more frequent in patients with Huntington's disease than in 39 control subjects, who had alterations of the echotexture in 12.8% (4/39) of the examinations. The number of CAG repeats and the clinical status correlated with the identification of hyperechogenic lesions of the substantia nigra (p<0.01). Hyperechogenic lesions of the caudate nucleus were associated with an increased signal intensity in T2 weighted MR images (p<0.05). All TCS parameters indicating brain atrophy correlated with CT findings (p<0.0001). CONCLUSIONS: TCS detects primarily abnormalities of the caudate nucleus and substantia nigra in Huntington's disease. These changes in the echotexture may represent degenerative changes in the basal ganglia matrix and are partially associated with CAG repeat expansion and the severity of clinical findings.     

Basal ganglia atrophy in prodromal Huntington's disease is detectable over one year using automated segmentation

Future clinical trials of neuroprotection in prodromal Huntington's (known as preHD) will require sensitive in vivo imaging biomarkers to track disease progression over the shortest period. Since basal ganglia atrophy is the most prominent structural characteristic of Huntington's pathology, systematic assessment of longitudinal subcortical atrophy holds great potential for future biomarker development. We studied 36 preHD and 22 age-matched controls using a novel method to quantify regional change from T(1) -weighted structural images acquired 1 year apart. We assessed cross-sectional volume differences and longitudinal volumetric change in 7 subcortical structures-the accumbens, amygdala, caudate, hippocampus, pallidum, putamen, and thalamus. At baseline, accumbens, caudate, pallidum, and putamen volumes were reduced in preHD versus controls (all P < .01). Longitudinally, atrophy was greater in preHD than controls in the caudate, pallidum, and putamen (all P < .01). Each structure showed a large between-group effect size, especially the pallidum where Cohen's d was 1.21. Using pallidal atrophy as a biomarker, we estimate that a hypothetical 1-year neuroprotection study would require only 35 preHD per arm to detect a 50% slowing in atrophy and only 138 preHD per arm to detect a 25% slowing in atrophy. The effect sizes calculated for preHD basal ganglia atrophy over 1 year are some of the largest reported to date. Consequently, this translates to strikingly small sample size estimates that will greatly facilitate any future neuroprotection study. This underscores the utility of this automatic image segmentation and longitudinal nonlinear registration method for upcoming studies of preHD and other neurodegenerative disorders.     

Basal ganglia calcifications in childhood

In the advent of widespread use of neuroimaging, calcification of the basal ganglia is visualized with increasing frequency. In this monograph, modern neuroimaging features of various disorders causing basal ganglia calcification in childhood are illustrated and discussed.     

Basal ganglia and thalamo-cortical hypermetabolism in patients with spasmodic torticollis

Introduction – The basal ganglia are thought to be involved in the primary dystonias, largely because of the repeated demonstration of neuropathological changes in these nuclei in the secondary dystonias. A hyperactivity of a network involving basal ganglia has been suggested in experimental animal dystonia. To test this hypothesis in humans, we studied the functional correlates of primary cervical dystonia using [¹⁸F]FDG and PET. Material and methods — Regional cerebral glucose metabolism (rCMRglc) was measured in 10 patients with idiopathic torticollis (6 drug-free and 4 drug-naive) and in 15 normal controls, using 2-[¹⁸F]-fluoro-2-deoxy-D-glucose ([¹⁸F]FDG) and positron emission tomography (PET). Results — A significant hypermetabolism in the basal ganglia, thalamus, premotor-motor cortex and cerebellum in the patients compared with normal controls was found. The patients were correctly assigned to their clinical category by a discriminant function analysis with a total accuracy of 96%. Conclusion — The results support the hypothesis that a dysfunction of a subcortical-cortical motor network may play a role in the pathogenesis of focal dystonia, in agreement with the experimental dystonia models.     

Basal ganglia and limbic system pathology in schizophrenia. A morphometric study of brain volume and shrinkage

The volume of several parts of the basal ganglia and of the limbic system was measured by planimetry of myelin-stained serial sections in postmortem brains of 13 schizophrenic patients and nine control cases. The medial limbic structures of the temporal lobe (amygdala, hippocampal formation, and parahippocampal gyrus) and the pallidum internum were significantly smaller in the schizophrenic group, whereas the pallidum externum showed only a modest trend toward volume reduction. The volumes of the putamen, nucleus caudatus, nucleus accumbens, and the bed nucleus of the stria terminalis did not differ between patients and controls. The volume reductions of the limbic temporal structures and of the pallidum internum of schizophrenics are interpreted as degenerative shrinkages of unknown etiology.     

Basal ganglia abnormalities in tardive dyskinesia

The purpose of the present study was to investigate CT abnormalities in tardive dyskinesia (TD) and to search for possible relationships with clinical data. A group of 30 psychotic patients (15 schizophrenic and 15 affective disorder) with TD was compared to a matched group of 30 psychiatric patients without TD and a matched group of 30 healthy controls. CT data were analyzed using two multivariate statistical methods [multidimensional scaling (MDS) and step-wise discriminant analysis]. MDS clearly separated both TD and non-TD groups from the healthy control group on the basis of CT parameters. Caudate left area reduction and left temporal sulci enlargement were the most important parameters that discriminated TD from non-TD patients. Only in TD-patients did caudate left area reduction and left temporal sulci enlargement correlate significantly with cumulative duration of psychiatric hospitalizations. The data of the present study support the findings of structural abnormalities in the caudate nucleus and in the temporal lobe of patients with TD. These abnormalities were especially marked in the left hemisphere. It is assumed that some factor related to longer psychiatric hospital treatment (e.g. neuroleptic intake) could account for these abnormalities.     

Basal ganglia activation in Parkinson's disease

The objective of this study was to compare basal ganglia activation in patients with Parkinson's disease to that of healthy controls, using functional MRI (fMRI). Six mildly-affected patients, off antiparkinsonian medications for at least 12h, and seven age-matched controls performed a unilateral motor switching task during fMRI data acquisition. Clear differences in basal ganglia activation were seen, with control subjects showing greater activation during both the left and right movement sessions. We observed activation of right sided basal ganglia structures in both groups, particularly with right sided movements, with caudate activation noted most frequently. This observation is consistent with right caudate involvement in the learning of new tasks and in association with externally paced movements.     

Basal ganglia calcifications in children]

We report the study of four children with bilateral basal ganglia calcifications (BGC) visualized on CT scan. Epilepsy was the clinical manifestation of three patients whose laboratory investigation revealed abnormal calcium metabolism. The first aim of this paper is to call attention to a treatable entity that can cause epileptic syndromes in infancy and childhood. The second purpose is to review the literature comparing with our fourth child who presented encephalopathy with BGC.