Quantitative spectral electroencephalography in predicting survival in patients with early Alzheimer disease.

OBJECTIVE: To determine whether measures of quantitative spectral electroencephalography (EEG) can predict survival in patients with early Alzheimer disease. DESIGN: Prospective cohort study; median duration of follow-up was 4.4 years in survivors and 2.6 years in nonsurvivors. Cox proportional hazards models, with adjustment for age and sex were used to estimate relationships between EEG measures and survival. Log relative percentage values of EEG bands were used as predictors. SETTING: Outpatient university memory clinic. PARTICIPANTS: One hundred one consecutively referred patients with early probable Alzheimer disease according to National Institute of Neurological and Communicative Disorders and Stroke-Alzheimer's Disease and Related Disorders Association criteria were studied with EEG at the time of diagnosis. The mean age of the patients was 79.2 years, which was higher than in previous EEG studies. MAIN OUTCOME MEASURE: Mortality. RESULTS: Fifty-one patients (50.5%) died during follow-up, with a median survival time in all patients of 4.1 years. The following EEG variables were significantly associated with increased risk of mortality: from parieto-occipital leads, higher theta (hazard ratio, 2.05; 95% confidence interval, 1.15-3.66; P<.05), lower alpha (hazard ratio, 0.43; 95% confidence interval, 0.25-0.76; P<.01), and lower beta (hazard ratio, 0.38; 95% confidence interval, 0.22-0.68; P<.001) activity; and from frontocentral leads, higher theta activity (hazard ratio, 2.07; 95% confidence interval, 1.17-3.66; P<.05). Stepwise Cox regression analysis showed that loss of parieto-occipital beta (P<.01) and alpha (P<.05) power were independent and significant predictors of mortality. Both beta (12.6-35.4 Hz) and alpha (7.5-12.5 Hz) activity remained significantly associated with mortality after adjustment for education, dementia severity, symptom duration, level of cognitive function, presence of extrapyramidal symptoms or hallucinations, presence of vascular risk factors, and presence of leukoaraiosis or local cortical atrophy. CONCLUSIONS: Decreases of beta and alpha activity on quantitative spectral EEG are independent predictors of mortality in patients with early Alzheimer disease. In the clinical context, the use of EEG technology for prediction of survival in individual patients remains to be determined.

Disequilibrium in patients with atherosclerosis: relevance of pontine ischemic rarefaction. Amsterdam Vascular Medicine Group.

OBJECTIVE: To examine the clinical relevance of isolated pontine hyperintense lesions (PHLs) on MRI in patients with atherosclerosis. METHODS: Seventeen atherosclerotic patients with isolated PHL on MRI were compared with 17 patients without PHL and were matched for age, sex, and initial manifestation of atherosclerosis. Subjects and observer were blinded to the MRI findings. We assessed symptoms, impairment, and disability with a structured interview and neurologic examination as well as disability scales. RESULTS: On all items, patients with PHL scored worse than did their controls. We found the largest differences in frequencies of symptoms of disequilibrium, difficulties with speech or swallowing, the Timed Walking Test, and the body care and movement subscale of the Sickness Impact Profile. Except for disequilibrium (p = 0.04), these differences did not reach statistical significance. Abnormal tandem-walking tests were more frequent in patients than they were in controls. Pyramidal signs were equally distributed. CONCLUSIONS: We propose PHL as a cause of symptoms of disequilibrium in patients with atherosclerosis. Symptoms are probably elicited by dysfunction of the corticopontine fibers, the pontocerebellar fibers, or the pontine nuclei.

rCBF-SPECT in brain infarction: when does it predict outcome?

We prospectively studied 26 patients with ischemic stroke within 24 hr, after 2 wk, and after 6 mo with thallium-201-diethyldithiocarbamate single-photon emission computed tomography (SPECT) and neurologic and functional assessments. The admission flow deficits correlated with outcome. The admission and 6-mo scores correlated with clinical conditions at each time. At 2 wk, the flow deficits were smaller and did not correlate with clinical parameters. Nor did the presence or absence of hyperfixation of the radiopharmaceutical. Six months after the infarct, the flow defect had decreased in 9 of 15 patients in whom three serial scans were available, with better clinical improvement than in the remaining six whose flow deficits increased. More patients in the first group had been treated randomly with the calcium-entry blocker flunarizine. SPECT imaging of rCBF within 24 hr after stroke correlates with clinical outcome and condition, whereas rCBF imaging at 2 wk after the stroke shows no clinical correlation.

Congenital anomalies in the teratological collection of Museum Vrolik in Amsterdam, The Netherlands. III: primary field defects, sequences, and other complex anomalies.

The Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Center, University of Amsterdam, founded by Gerardus Vrolik (1775-1859) and his son Willem Vrolik (1801-1863), consists of more than 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, the collection of congenital anomalies, comprising 360 specimens, was recatalogued and redescribed according to contemporary morphogenetic views. The original descriptions, if preserved, were compared with the clinical, radiographical, and magnetic resonance imaging findings. In 120 specimens the following midline anomalies, primary field defects, and sequences were diagnosed: holoprosencephaly sequence; aprosencephaly/atelencephaly; cleft lip; Pierre Robin sequence; omphalocele; diaphragmatic hernia; ectopic heart; bladder exstrophy; cloacal exstrophy; caudal dysgenesis; prune belly sequence; sirenomelia; fetal akinesia sequence; and disruption sequences. Polytopic field defects and associations were diagnosed in 25 specimens. We discuss the opinions of Gerardus and Willem Vrolik, who tried to find reasonable explanations for the dysmorphogenetic entities they investigated, rather than accepting the traditional superstitions.

Congenital anomalies in the teratological collection of Museum Vrolik in Amsterdam, The Netherlands. V: conjoined and acardiac twins.

The Museum Vrolik collection of the Department of Anatomy and Embryology of the Academic Medical Center, University of Amsterdam, founded by Gerardus Vrolik (1775-1859) and his son Willem Vrolik (1801-1863), consists of more than 5,000 specimens of human and animal anatomy, embryology, pathology, and congenital anomalies. Recently, the collection of congenital anomalies, comprising 360 specimens, was recatalogued and redescribed according to contemporary perspectives. The original descriptions, as far as preserved, were compared with the clinical, radiographic, and magnetic resonance imaging findings. We diagnosed 30 symmetrical conjoined twins (CTs), 11 parasitic CTs, and 16 acardiac twins. Within the group of symmetrical CTs, the following concomitant external anomalies were found in 15 specimens: neural tube defects, holoprosencephaly, cleft lip/palate, umbilical hernia, omphalocele, cloacal exstrophy, peromelia, polydactyly, and facial abnormalities suggestive of a chromosomal abnormality. We discuss the results in the light of historical and contemporary explanations regarding conjoined twinning, including the opinions of Gerardus and Willem Vrolik and of Louis Bolk, one of their successors.

A 100-year-old anatomical specimen presenting with boomerang-like skeletal dysplasia: diagnostic strategies and outcome.

The Museum Vrolik collection of human anatomy comprises 360 recently redescribed specimens with congenital anomalies. The specimen described here dated from 1881 and presented with a general embryonic appearance, disproportionate short stature, brachycephaly, widened cranial sutures, hypertelorism, microphthalmia, bilateral cleft lip and palate, micrognathia, short and curved limbs, polysyndactyly, and abnormal female genitalia. Conventional radiography was hampered by decalcification of the skeleton, due to acidification of the preservation fluid. The use of additional imaging techniques, i.e., mammography, computerized tomography with three-dimensional reconstruction, and magnetic resonance imaging eventually led us to conclude that the condition of our specimen was similar to Piepkorn type skeletal dysplasia, boomerang dysplasia, and a condition described by Carpenter and Hunter [1982: J Med Genet 19:311-315], though none of these diagnoses seemed fully applicable.

Sideways displacement and curved path of recti eye muscles.

We investigated the sideways displacement of recti muscles with the eye in various gaze-positions by making computed tomographic (CT) scans in a plane perpendicular to the muscle cone, posterior to the globe. We found no consistent sideways displacement of the horizontal recti in the up or down gaze or the vertical recti in the left or right gaze. We found a substantial displacement, however, of the contracting and relaxing muscles. Consequently, we made CT scans in a horizontal plane through medial and lateral recti and found that, with the eye in primary position, the recti muscles follow a curved path. The curvature of the path increased following muscle relaxation and decreased following muscle contraction.

Recovery after central pontine myelinolysis.

Central pontine myelinolysis (CPM) is known to be almost universally fatal. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. We present a case in which the diagnosis of CPM was confirmed by computed tomography (CT); the patient had two symptoms not previously reported and made a remarkable recovery.

Jaw movement responses to electrical stimulation of different parts of the human temporalis muscle.

Previous EMG studies have provided indications for the differential activation of the human temporalis muscle. However, in these studies the contribution of different parts of the temporalis muscle could not be separated from the contributions of other muscles, since contraction was performed by voluntary activation. Therefore, the aim of this study was to examine functional differences among various parts of the human temporalis muscle by means of recording the incisal point movement response to electrical stimulation of parts of the muscle. Five healthy male subjects participated in this study. Three locations (anterior, middle, and posterior temporalis muscle) were stimulated, by means of monopolar wire electrodes and rectangular pulses. The insertion depths of the stimulation electrodes were determined by means of magnetic resonance images. Stimulation was performed in four jaw positions (resting position, 50% maximum mouth opening, 1 cm to the left, and 1 cm to the right). Movement responses to stimulation of the different muscle parts were recorded with the OKAS-3D jaw movement analysis system. The movement responses were expressed in polar coordinates. The variation in the direction of the jaw movement response was partly explained by the factors 'stimulation location' and 'jaw position' (ANOVA, p < 0.001). When the stimulation location shifted in an antero-posterior direction, the response changed from a vertical-lateral incisal point movement to a lateral-posterior movement with a smaller vertical component. The jaw position during stimulation also influenced the movement response. A functional subdivision of the temporalis muscle into at least three parts is favored.

Epidermal nevus syndrome with isolated enlargement of one temporal lobe: a case report.

A 5-month-old girl presented with infantile spasms and psychomotor retardation. She was born with epidermal nevi on the right side of the head, the right arm and the back. MRI of the head showed a right hemimegalencephaly limited to the temporal lobe. There were no MRI signs of neocortical dysplasia. Ultrasonography revealed multiple cysts in both ovaria and a small solid lesion in the left adrenal gland. Hemimegalencephaly represents an overgrowth phenomenon; the isolated temporal lobe hypertrophy can be explained as a mild expression of hemimegalencephaly. To our knowledge this has not been described before.

Tuberous sclerosis: the incidence of sporadic cases versus familial cases.

In 48 families in which tuberous sclerosis occurred, extensive examination presented almost the same incidence of sporadic cases as reported in previous studies. Although inspection of the skin and cranial computed tomography seem to be the most sensitive diagnostic tests available, negative results with these methods do not exclude the diagnosis. Estimation of alpha 2-macroglobulin serum level does not mean an extension of the diagnostic arsenal.

Perineural tumor extension along the trigeminal nerve: magnetic resonance imaging findings.

OBJECTIVE: To evaluate the magnetic resonance imaging (MRI) findings of 15 patients with perineural tumor extension along the trigeminal nerve in correlation with clinical data. METHODS: The clinical records and MRI studies of 15 patients with perineural tumor extension along the trigeminal nerve were retrospectively reviewed. Imaging studies included plain and contrast-enhanced thin section T1-weighted spin echo (T1-WSE) MRI with and without fat-suppression. The studies were compared to determine which sequence provided greatest tumor conspicuity and best depiction of tumor extent. The conspicuity of these tumors was assessed on the available sequences by two observers by consensus. RESULTS: The contrast-enhanced T1-weighted spin echo fat-suppressed images (T1-WSECEFS) demonstrated greatest tumor conspicuity and best depiction of tumor extent in the extracranial head and neck and skull base region. The conventional T1-weighted spin echo pre- and postcontrast images were, however, diagnostic of perineural tumor extension in 11 patients due to the presence of considerable tumor bulk and extension well above the skull base. In the other four patients the perineural tumor was poorly visualized on the conventional T1-WSE images and well visualized on the fat-suppressed images. The mandibular division of the trigeminal nerve (V3) was most commonly involved (n = 10), followed by the maxillary (V2; n = 5) and ophthalmic (V1; n = 2) division. Two patients had both mandibular as well as maxillary nerve involvement. The finding of perineural tumor extension had significant impact on patient management: based on the MR imaging study, the primary tumor was considered inoperable (n = 13), the extent of surgery was expanded (n = 2) and radiation therapy (RT) ports were extended (n = 12). CONCLUSION: Complete trigeminal nerve imaging is recommended when evaluating (suspected) head and neck malignancies with a high risk for perineural extension. In these cases thin section axial and coronal precontrast T1-WSE MR images and postcontrast T1-WSE MR images with fat-suppression should be obtained. In the rare event that artifacts degrade the quality of the fat-suppressed images, contrast-enhanced T1-WSE sequences without fat-suppression can additionally be used.

Mental changes in subcortical arteriosclerotic encephalopathy.

We describe six patients with chronic hypertension, atherosclerotic vascular disease and periventricular leukoencephalopathy (PVL) on CT. PVL was combined with cortical infarcts in one and with lacunar infarcts in five patients. On neuropsychological examination all patients showed the characteristic features of a subcortical type of mental deterioration. Clinico-radiologic correlation suggests a direct association of PVL and subcortical dementia, and it is argued that prominent cortical features in the dementia of patients with vascular disease and PVL suggest the concomitant presence of Alzheimer's disease.

Progressive multifocal leukoencephalopathy in a case of acquired immune deficiency syndrome.

The case of a 40-year-old homosexual male with A.I.D.S. (Acquired Immune Deficiency Syndrome) and P.M.L. (Progressive Multifocal Leukoencephalopathy) is described. The importance of a brain biopsy for diagnostic procedures, especially in the case of a patient with A.I.D.S. is stressed. The diagnosis P.M.L. has been made by means of light- and electronic microscopical examination, and the presence of JCV-DNA in the brain tissue has been confirmed by dot hybridization. Various antiviral treatments did not show any effect on the course of the P.M.L.

Functional brain imaging with I123-amphetamine. First experience in the Netherlands.

Single Photon Emission Computed Tomography (SPECT) has been used in the last five years as a method for cerebral bloodflow imaging, especially in cerebral infarction. In this study the first experiences in the Netherlands are presented. In 57.6% of our patients lesions, defined by SPECT were larger than those found by CT. This was not only seen in patients with cerebral infarction but also in hematoma. In 33.3% the size of the lesions were comparable. In 10 out of 14 patients with a solitary lesion in one hemisphere a decreased perfusion was seen in the contralateral cerebellar hemisphere. This phenomenon is called crossed cerebellar diaschisis. One patient with probably Alzheimer disease, showing a specific flow pattern, is discussed.

Focus localization in patients with partial epilepsy with 99Tcm-HMPAO SPECT under continuous surface EEG monitoring.

Our study was aimed at determining the validity of 99Tcm-HMPAO single photon emission computed tomography (SPECT) in the localization of the epileptogenic focus by correlating this diagnostic method with other auxiliary investigations such as surface EEG and X-ray transmission computed tomography (CT). Twenty-eight patients with partial epilepsy and spontaneous memory complaints were assessed with a 21-channel EEG recorded during psychometric evaluation of the memory complaints and with a 12-channel EEG during 99Tcm-HMPAO SPECT. No significant agreement was observed for total focus localization by the two EEGs, CT or SPECT. The EEGs were highly reproducible. Frontal and temporal pathology on CT resulted in frontal and temporal cold spots on SPECT on the same side in six patients. In addition, SPECT demonstrated frontal, temporal or posterior temporal-inferior parietal cold spots in nine patients without accompanying pathology on CT. It is concluded that CT and SPECT may provide complementary anatomical and functional information, respectively, to the EEG. Furthermore, SPECT may be complementary to CT in visualizing functional deficits without an anatomical correlate. The value of SPECT studies in partial epilepsy may be improved by increasing the resolution of the SPECT system and by simultaneous EEG monitoring.

Memory complaints, memory disorders and focus localization in patients with partial epilepsy.

Our study aimed at analysing effects of epileptic foci on memory function in patients with partial epilepsy. Twenty-eight patients with spontaneous memory complaints and psychometrically established memory disorders were assessed by 21-channel electroencephalography recorded both during cognitive testing and during 99mTc-HMPAO single photon emission computed tomography (SPECT). Computed tomography (CT) was performed on the same day. None of the epilepsy-related factors (seizure type, seizure frequency, type of epilepsy, age at onset of the seizures, type of antiepileptic treatment) could be related directly to severity or type of memory impairment (classified into the categories 'global', 'verbal' and 'non-verbal'). Remarkably, this study found no significant relationship between EEG focus localization and severity of measured memory impairment. Most areas with hypoperfusion on the SPECT were found in the group with global (severe) amnesia, typically with a right frontal localization. Abnormalities on CT were predominantly found in the same group, however, with a right-sided parietal localization. An unanticipated finding was that the majority of temporal CT and SPECT lesions were found in the group with relatively better memory performance.

[Wilson's disease; diagnosis with the aid of magnetic resonance tomography]. / Ziekte van Wilson; ontmaskering met behulp van kernspinresonantie-tomografie.

In a 21-year-old woman with a smaller and sloppy handwriting, drooling especially when stooping, sporadic choking, clumsiness, and frequent stumbling, Wilson's disease was diagnosed. The medical history disclosed a short period of haemolytic anaemia with transient hepatic failure, and irregular menstruation periods with infertility. On examination there were no signs of liver or spleen enlargement. She was slow, had an expressionless face and mild dysarthria, and slight impairment of the coordination of the limbs. Magnetic resonance imaging of the brain showed bilateral hyperintensive lesions of the basal ganglia on T2W images. Zinc therapy induced a good biochemical response and there was also some clinical improvement. Linkage analysis within the family identified one other asymptomatic homozygotically affected sister. A diagnostic delay occurs frequently due to relative unfamiliarity with this rare disease and due to its variable clinical expression.