Severe myoclonic epilepsy in infancy: evolution of seizures

Demonstration of the full extent of abnormality in patients with the Struge-Weber syndrome (SWS) is important for prognosis and in planning surgery to remove the seizure focus. We compared single-photon emission computed tomography (SPECT), MRI and CT in nine children under the age of 4 years with seizures as part of SWS, in an attempt to determine the optimal method of imaging in different clinical settings. Seven unilateral and two bilateral cases were studied by interictal 99mtechnetium hexamethylpropyleneamineoxime (HMPAO) SPECT, and contrast-enhanced CT and MRI, giving information on 11 abnormal hemispheres. All imaging modalities showed abnormalities in every child. Perfusion imaging showed focal regions of decreased uptake in 9 of 11 (82%) abnormal hemispheres and demonstrated a widespread decrease but no focal defect in 2; it also revealed crossed cerebellar diaschisis in 2 cases. CT demonstrated typical gyriform calcification in 9 of 11 (82%) affected hemispheres. Contrast-enhanced MRI showed more extensive involvement than contrast-enhanced CT in 5 of 11 (45%) cases. The area of hypoperfusion shown by SPECT was smaller than the area of contrast enhancement on MRI in 6 of 11 cases (55%), comparable in 3 (27%) and larger in 2 cases (18%). CT is sufficient to confirm the clinical diagnosis of SWS, but MRI frequently shows more extensive abnormal areas. 99mTc HMPAO imaging is a useful addition when it is important to know the full extent of the disease, for example prior to surgery. It is likely to detect areas of hypoperfusion, representing ischaemic regions, which may act as an epileptogenic focus and may not be shown by CT or MRI.     

Unilateral saccadic pursuit in patients with sensory stroke: sign of a pontine tegmentum lesion

BACKGROUND AND PURPOSE: Pure hemisensory syndrome can be caused by small strokes occurring in a number of regions, including the thalamus and pons. Differentiation of the pontine sensory syndrome from the thalamic sensory syndrome has generally been made on the basis of distribution of sensory loss and involvement of specific sensory modalities but not without uncertainties and difficulties. Because the pontine tegmentum plays a pivotal role in generating horizontal eye movement, we attempted to discriminate these 2 syndromes by analyzing horizontal eye movements in stroke patients with pure hemisensory syndrome. METHODS: Horizontal saccade, pursuit, vestibulo-ocular reflex (VOR), and VOR cancellation (VORC) were evaluated using electro-oculography in 6 patients with hemisensory syndromes, 3 due to pontine stroke and 3 due to thalamic stroke, and all were verified by MRI or CT. In addition, somatosensory evoked potentials (SEPs) were recorded. RESULTS: Smooth pursuit and VORC directed toward the side of the lesion were impaired unilaterally in patients with pontine sensory stroke, whereas those 2 movements were intact bilaterally in patients with thalamic sensory stroke. Saccade and VOR were preserved in all patients. SEPs were normal in all patients with pontine and thalamic sensory strokes. No difference was found in the pattern of sensory disturbance between the 2 types of stroke patients. CONCLUSIONS: Ipsilateral impairment of the smooth pursuit system may be a sign of a pontine lesion in patients with hemisensory stroke.     

The effect of different porcelain conditioning techniques on shear bond strength of stainless steel brackets

A 51-year-old man was admitted because of bilateral fatigable blepharoptosis with diurnal fluctuation. He had a tendency to take much instant foods before onset. On admission, he showed symmetrical bilateral ptosis, supranuclear upward gaze palsy and vertical diplopia. Ptosis mildly improved on lateral or upward gaze. Bell's phenomenon and pupillary response were intact. Intravenous edrophonium infusion test and serum antiacetylcholine receptor antibody were negative. Cranial MRI and CT demonstrated the lesion in the periaqueductal gray (PAG) region from superior colliculus to upper pontine level, which was remarkably enhanced by Gd-DTPA infusion. He was diagnosed as midbrain ptosis, probably due to atypical Wernicke's encephalopathy. It was conceivable that the PAG lesion might be contributory to fatigable blepharoptosis (pseudomyasthenia) and supranuclear upward gaze palsy in the present case, because the PAG controls levator palpebrae neurons of central caudal nucleus in oculomotor nucleus complex and receives afferents from the limbic system., reticular formation and posterior commissure.     

Ipsilateral oculomotor nerve palsy and contralateral downbeat nystagmus due to unilateral paramedian thalamopenduncular infarction--a case report

A case of midbrain and thalamic infarction which showed complete oculomotor nerve palsy of the ipsilateral eye and monocular downbeat nystagmus, ptosis, upward movement disturbance and adduction disturbance of the contralateral eye was reported. The patient is a 53-year-old woman who was admitted to our hospital because of sudden onset of double vision. Head magnetic resonance imaging showed unilateral midbrain and thalamic infarction. The midbrain lesion was located in the paramedian area and the bilateral ptosis, bilateral upward gaze palsy and adduction disturbance of the contralateral eye were considered to be caused by the lesion involving the unilateral oculomotor nucleus. This case is considered to be important because the association of contralateral monocular downbeat nystagmus is very rare.     

A genetic propensity to high factor VII is not associated with the risk of myocardial infarction in men

Interictal brain SPECT is useful for the localization of a seizure focus. Concomitant hypoperfusion of the ipsilateral thalamus on interictal SPECT has been noted for temporal lobe epilepsy. In this study, we aimed to evaluate the prevalence of thalamic hypoperfusion ipsilateral to temporal hypoperfusion (ipsilateral thalamic hypoperfusion) and to assess the usefulness of this finding for the lateralization of epileptic foci on interictal SPECT for temporal lobe epilepsy patients. METHODS: Forty-six patients with refractory temporal lobe epilepsy underwent interictal brain SPECT after intravenous injection of 555-740 MBq of 99mTc-ECD. Perfusion impairments in the brain, especially the temporal lobe and thalamus, were evaluated. The localization of seizure foci was determined in conjunction with scalp, ictal and cortical electroencephalography, MRI and clinical outcomes. Ictal SPECT was performed for 5 of the 12 patients. RESULTS: Concomitant decreased perfusion in both the temporal lobe and the ipsilateral thalamus was observed for 12 (26%) of 46 temporal lobe epilepsy patients on interictal brain SPECT. Seven patients showed hypoperfusion in the left temporal lobe and ipsilateral thalamus. Five patients showed hypoperfusion in the right temporal lobe and ipsilateral thalamus. In addition, hypoperfusion in the ipsilateral basal ganglia (ten patients) or contralateral cerebellum (four patients) was observed. CONCLUSION: Ipsilateral thalamic hypoperfusion is not uncommon in temporal lobe epilepsy. The exact mechanism causing ipsilateral thalamic hypoperfusion is uncertain; however, corticothalamic diaschisis may be an important factor. This finding may aid in the lateralization of seizure foci on interictal brain SPECT.     

MRI-radiofrequency tissue tagging in patients with aortic insufficiency before and after operation

We examined the relation between language dominance and regional cerebral blood flow (rCBF) during the intracarotid amobarbital procedure (IAP). A previous report limited to three patients suggested that dominant rather than nondominant hemisphere IAP may have a differential effect on rCBF. Behavioral assessment during the IAP also suggests that dominant hemisphere injection results in a differential effect on memory and affective symptoms rather than nondominant injection. Thirteen patients were assessed using single-photon emission CT (SPECT) brain imaging during both left and right IAP. The SPECTs were coregistered with the individual's MRI. Changes in rCBF during each IAP were compared with the patient's baseline SPECT. Nine patients had left hemisphere dominance, two were right dominant, and two had bilateral speech representation. In the left dominant subjects, left-hemisphere injection had a consistently greater effect on rCBF than right-hemisphere injection in the anterior (p < 0.005) and posterior (p < 0.01) temporal neocortex. There was also a trend for greater hypoperfusion in the frontal lobe of the left hemisphere. rCBF in the ipsilateral hippocampus was not significantly different after each injection (p > 0.05). In the two patients with right hemisphere speech, the reverse pattern was seen, with greater hypoperfusion after right (dominant) hemisphere injection. There was no consistent asymmetry in the two patients with bilateral speech. Dominant hemisphere IAP results in significantly greater hypoperfusion than does nondominant injection. These data provide a physiologic basis for behavioral differences noted after dominant versus nondominant IAP.     

Photodynamic therapy for early stage squamous cell carcinoma of the lung

We studied 9 patients with bilateral vertebral artery occlusion (BVAO). BVAO was confirmed using angiography in order to clarify its clinical feature, mechanism, and long term prognosis. Three patients showed bilateral intra-cranial occlusion, 3 bilateral extra-cranial occlusion, and 3 intra and extra-cranial occlusion. The basilar artery was fed by the posterior communicating artery in 8 out of 9 patients. In one of the 8, reconstitution of the thyrocervical artery was seen. We divided the patients into 4 groups according to MRI findings, as follows: Group 1 with no abnormal finding on MRI (N = 2); Group 2 with deep pontine infarcts and non-territorial small cerebellar infarcts (N = 2); Group 3 with extended pontine infarcts (N = 3); and Group 4 with cerebellar cortical artery infarcts, deep pontine infarcts, and non-territorial small cerebellar infarcts (N = 2). Transient episodes were seen in all patients, 8 patients out of 9 had vertigo/dizziness, 3 tinnitus, 2 diplopia, 2 headache, 2 numbness, and 1 hearing disturbance. These episodes preceded a final attack or complete stroke 2 days to 5 months, and those who had a longer period of episodes in the preceding term tended to have less severe deficits. Six of the patients had vertebro-basiler symptoms after being in the upright position, including all the patients in Groups 2 and 4, which had cerebellar border zone/terminal zone infarcts. These results indicate that the hemodynamic mechanism plays an important role in BVAO. The prognosis was not always grave. Four of the patients could walk independently, 2 could walk with a cane, and 3 were bed ridden (2 of which died). Long-term follow-up data (a mean of 5 years) were obtained in all patients. In the patients who could walk, one had asymptomatic cerebellar infarcts, and one had TIAs frequently. Patients with BVAO often also have TIAs and/or preceding episode and show cerebellar border zone/terminal zone infarcts. This research strongly suggests that hemodynamic mechanism might play an important role in BVAO, and that paying attention to border zone infarction in MRI and transient episodes can lead to earlier diagnosis and treatment.     

Carboxylic ester hydrolase and amylase in ischemic pancreatitis in the guinea pig

OBJECTIVES: To define the clinical, neuropsychological, and radiological features of bilateral parietal lobe atrophy. METHODS: Four patients underwent a comprehensive longitudinal neuropsychological assessment, as well as MRI and HMPAO-SPECT. RESULTS: The consistent findings in the patients were early visuospatial problems, agraphia of a predominantly peripheral (or apraxic) type, and difficulty with bimanual tasks, all of which outweighted deficits in memory and language until later in the course of the illness. As the disease progressed, impairments in the phonological aspects of language and in auditory-verbal short term memory were often striking, perhaps reflecting spread from the parietal lobe to perisylvian language areas. Three patients went on to develop a global dementia and fulfilled the criteria for a clinical diagnosis of probable Alzheimer's disease; the fourth patient has only recently been identified. Neuroimaging disclosed bilateral parietal lobe atrophy (MRI) and hypoperfusion (SPECT), which was out of proportion to that seen elsewhere in the brain. One patient has died and had pathologically confirmed Alzheimer's disease with particular concentration in both superior parietal lobes. CONCLUSIONS: Bilateral biparietal atrophy is a recognisable clinical syndrome which can be the presenting feature of Alzheimer's disease. Although the label "posterior cortical atrophy" has been applied to such cases, review of the medical literature suggests that this broad rubric actually consists of two main clinical syndromes with features reflecting involvement of the occipitotemporal (ventral) and biparietal (dorsal) cortical areas respectively.     

Delayed onset generalised dystonia after cyanide poisoning

A 27 year old female developed delayed onset of persistent generalized dystonia following a suicidal attempt with potassium cyanide. Cranial CT scan showed bilateral putaminal hypodensities which were also seen on MRI scans to be hypointense on T1 and hyperintense on T2 weighted images. Multimodality evoked potentials were normal. An improvement was noted with levodopa.     

Sickle cell anemia and internal cerebral vein thrombosis

Cerebrovascular disorders are frequently associated with sickle cell disease, mainly in homozygous children. We report the case a 25-old-patient with known sickle cell disease who presented with coma inaugurated by manifestations of intracranial hypertension. CT revealed bilateral thalamic infarcts and angiography confirmed the thrombosis of internal cerebral veins. Treatment included heparin and blood transfusion. Severe cerebral oedema resulted in the lethal outcome three days later.     

Single-photon emission CT findings in acute Japanese encephalitis

PURPOSE: To determine the usefulness of single-photon emission CT (SPECT) in the diagnosis of acute Japanese encephalitis (JE). METHODS: We examined 10 patients (six men and four women; mean age, 69 years) with viral encephalitis. We divided the cases into two groups: the JE group (n = 4) and the non-JE group (n = 6; two with herpes simplex encephalitis and four with encephalitis of unknown origin). All cases were investigated with 99mTc-hexamethylpropyleneamine oxime (HMPAO) SPECT within 15 days after the onset of symptoms. Two patients in the JE group were also examinated by SPECT at a later stage. In all cases MR imaging was performed after the SPECT study. RESULTS: In the acute stage, all patients in the JE group showed a marked increase of HMPAO uptake that matched the hyperintense area observed on MR images in the thalami and putamina bilaterally. Follow-up SPECT studies of two patients with JE revealed a decrease of HMPAO deposition in the areas of high uptake. None of the patients in the non-JE group had an increased accumulation of HMPAO in the thalami or the putamina. CONCLUSIONS: SPECT is helpful in differentiating JE from herpes simplex encephalitis and other types of encephalitis. SPECT may be useful as a diagnostic tool in the early stages of JE.     

Symptomatic third ventricular choroid plexus cysts

We describe the imaging findings in 3 children with choroid plexus cysts (CPC) at the foramen of Monro. All CPC measured less than 2 cm and produced symptoms of raised intracranial pressure when located at the foramen of Monro where there was obstruction to the cerebrospinal fluid (CSF) flow. Among the imaging studies done on our patients, CPC were depicted best by cranial sonography and CT-ventriculography and missed by standard CT and MRI. Misdiagnosis can lead to inappropriate shunting with adverse effects. Two of our patients had relief of symptoms after resection of the cyst. One patient with inoperable cardiac defects died and had no surgery performed. Serial CT and sonographic studies in this patient showed progression in the size of the cyst and ventriculomegaly. Cranial sonography and CT-ventriculography are the modalities of choice in evaluating ventriculomegaly when the diagnosis of occult obstructive CPC is entertained in children. Cranial sonography is indicated in infants with an open fontanelle and CT-ventriculography is reserved for older children with hydrocephalus which is not responding to shunting.     

Nasopharyngeal carcinoma with intracranial spread: CT and MR characteristics

PURPOSE: Nasopharyngeal carcinoma (NPC) frequently spreads intracranially. We compare CT and MRI in identifying intracranial spread and reexamine the route of infiltration. METHOD: One hundred fourteen consecutive patients with proven NPC were evaluated prospectively with T1-, T2-weighted, contrast-enhanced MRI and CT. RESULTS: MRI showed 35 (31%) patients with middle cranial fossa involvement. Twenty-nine (25%) patients had cavernous sinus infiltration, while six (5%) showed only dural thickening. The most common route of spread is through the foramen ovale (FO) (12/35 patients, 34%), followed by skull base destruction (6/35 patients, 17%), foramen lacerum (FL) (6/35 patients, 17%), sphenoid sinus (6/35 patients, 17%), and combined FO and FL (5/35 patients, 14%). Using MRI as a standard, CT demonstrated the following involvement: cavernous sinus in 26 of 29 (90%) patients, FO in 9 of 12 patients, skull base in 6 of 6 patients, FO and FL in 3 of 5 patients, FL in 6 of 6 patients, sphenoid sinus in 6 of 6 patients and dura in 0 of 18 patients. CONCLUSION: It is believed that NPC most commonly spreads intracranially via the FL or by direct erosion. Perineural spread through the FO is an important route, which explains why with CT evidence of cavernous sinus involvement there may be no skull base erosion. These findings are best seen on MRI.     

Glial tumourettes (glial microtumours): their clinical and histopathological manifestations

This study represents our experience with eight cases (males: 4; females: 4; 13-47 years old, average age 28.5 years) of a "glial tumourette" (minute glioma), which measured less than 15 mm in diameter on an MRI. Four tumours were located in the frontal lobe, one in the rostrum of the corpus callosum, two in the midbrain, and one in the thalamus. The symptoms and signs lasted from two days to 15 months prior to diagnosis, and they consisted of epileptic seizures in five patients and increased intracranial pressure due to hydrocephalus resulting from aqueductal stenosis in three. All patients had a CT scan and an MRI as a part of their initial neuroimaging evaluations. While the CT findings failed to show the lesion in four patients, MRI demonstrated it in all cases. Five tumours were either totally or subtotally removed while the remaining three were biopsied. Histological examinations revealed six tumours to be low-grade gliomas (fibrillary astrocytoma: 4; oligoastrocytoma: 2) and two to be high-grade gliomas (anaplastic astrocytoma: 1; anaplastic oligodendroglioma: 1). Regarding adjuvant therapy, three patients received radiation and/or chemotherapy. One of the patients with midbrain fibrillary astrocytoma died of the disease 38 months after the operation, however, no evidence of progression in the remaining seven has been observed in the follow-up period ranging from five to 65 months after the operation (average: 25.4 months). The histogenesis of benign and malignant gliomas and the importance of surgical exploration in the management of such patients with minute intracerebral tumours are also discussed.     

Cerebral hypoperfusion during acute Kawasaki disease

BACKGROUND AND PURPOSE: Kawasaki disease is a febrile disease of children notable for systemic vasculitis. There have been many previous reports of various complications, including disorders of the central nervous system. We evaluated cerebral perfusion during the acute stage in patients with Kawasaki disease. METHODS: Single-photon emission-computed tomography (SPECT) with 99mTc-hexamethylpropyleneamine oxime was performed in 21 children with acute stage Kawasaki disease. Follow-up SPECT and MRI were performed about 1 month after the first SPECT in patients who exhibited abnormal SPECT findings during the acute stage. RESULTS: In 6 of 21 children SPECT imaging demonstrated localized cerebral hypoperfusion without abnormal neurological findings or clinical symptoms, and the follow-up SPECT and MRI approximately 1 month after the first SPECT revealed no abnormalities. CONCLUSIONS: Some patients with Kawasaki disease have transient localized cerebral hypoperfusion at the acute stage.     

Mechanism of action of a new component of the Ca(2+)-dependent proteolytic system in rat brain: the calpain activator

OBJECTIVE: Using single photon emission computed tomography (SPECT) we evaluated the presence and evolution of changes in brain perfusion in juvenile systemic lupus erythematosus (JSLE). METHODS: SPECT was performed in 14 patients with active JSLE divided in 2 groups: the first included 7 patients without central nervous system (CNS) involvement and the second 7 patients with minor neuropsychiatric symptoms (headache, reactive depression, cognitive impairment, mood swing). SPECT findings were compared to seroimmunological and magnetic resonance imaging (MRI) data. After 6 month followup, a second SPECT scan was performed in 12 of 14 patients. RESULTS: At baseline, SPECT showed perfusion defects in 2 patients without neuropsychiatric symptoms and in 5 patients with CNS involvement. In one of the 7 patients with altered SPECT, MRI showed focal hyperintensities. MRI alterations were observed in another patient who had a normal SPECT scan. Cortical atrophy was present in 5 of 14 patients. Correlation between neuropsychiatric manifestations and SPECT findings was not clearly evident because the major part of JSLE patients with CNS involvement and with SPECT alterations had multiple symptoms, but showed focal hypoperfusion on SPECT imaging. No significant association was found between seroimmunological data and SPECT findings. At followup, improvement of perfusion alterations was observed in 6 of 7 patients with altered SPECT and, in 3 of them, findings might be attributed to changes in steroid treatment. CONCLUSION: Perfusion abnormalities in SLE may represent reversible lesions or subclinical CNS involvement. Moreover, SPECT imaging appears to be useful in detecting and monitoring CNS involvement in SLE.     

Reproducibility of the acetylene rebreathe technique for determining cardiac output

Acute and subacute extrapyramidal movement disorders are rarely reported in uremic patients. We report three such cases with basal ganglia lesions. All three had advanced renal failure with high serum creatinine levels. One of the patients had a history of ischemic heart disease and acute pulmonary edema with hypoxemia. Another patient had experienced arterial hypotension during previous hemodialysis. The third had prominent metabolic acidosis. One of the patients developed generalized dyskinesias, whereas the other two developed gait disturbances. Neuroimaging studies in all three cases showed bilateral changes in the basal ganglia. The natural history was self-limiting with gradual improvement. Diminution of the basal ganglia lesions was demonstrated on follow-up imaging in two of the three cases. We conclude that acute or subacute movement disorders with bilateral basal ganglia lesions may occur in uremia. Hypoperfusion with global brain ischemia and selective vulnerability of the basal ganglia to uremic toxins may account for these lesions.     

Infantile familial cardiomyopathy due to mitochondrial complex I and IV associated deficiency

Two brothers, aged 27 and 20 months, born from consanguineous healthy parents, presented with cardiomyopathy, lactic acidosis and carnitine abnormalities in serum and muscle, without clinical evidence of muscle involvement. The histochemical reaction for cytochrome c oxidase (COX) activity was negative in all muscle fibres, although the holoenzyme and subunits were present at a normal level, as shown by immunocytochemistry. The COX activity was, respectively, 5 and 25% of control values, in muscle biopsies. Partial deficiency of complex IV was confirmed in fresh isolated muscle mitochondria from patient 2 and was associated with a defect of complex I. Patient 1 died at age 3 yr 6 months. Partial improvement of cardiomyopathy in patient 2 was obtained under carnitine therapy, but seizures occurred and CT scan and magnetic resonance imaging (MRI) revealed thalamic hypodensity. Thus, the disorder appears to be progressive despite the clinical stabilization of the cardiomyopathy. This further demonstrates the complexity and clinical heterogeneity of combined respiratory chain complex deficiencies.     

Daily variations in pineal melatonin concentrations in inbred and outbred mice

Magnetic resonance imaging (MRI), single photon emission computed tomography (SPECT) and magnetic resonance spectroscopy (MRS) were successively recorded in a 3-year-old girl with the acute hemiplegia syndrome. She was admitted to our hospital with complaints of fever, loss of consciousness and right side dominant clonic convulsions evolving into status epilepticus, and then recovered with sequelae of aphasia and right hemiparesis. Electroencephalography showed a generalized slow rhythm at the onset, and very low activities on the left hemisphere in the follow-up records. Brain CT and MRI revealed edema of the left hemisphere initially, followed by left side dominant brain atrophy. No cerebral vascular lesion was detected by magnetic resonance angiography. N-Isopropyl-[123I]-iodoamphetamine SPECT showed marked hypoperfusion of the left hemisphere accompanied by crossed cerebellar diaschisis. MRS at the initial stage detected decreased N-acetyl-aspartic acid and increased lactic acid signals in the bilateral hemisphere, which subsequently normalized only on the right side. These findings suggested brain damage and neural cell death in the left cerebral hemisphere, caused by acute encephalopathy. SPECT and MRS are useful new techniques to study the pathophysiology of the acute hemiplegia syndrome.     

Bilateral pheochromocytomas with von Hippel-Lindau's disease: a case report

A case of bilateral pheochromocytomas with von Hippel-Lindau's disease (VHL) is reported. A 46-year-old woman who had hyperglycemia was admitted to our hospital because of abdominal tumors. Her elder sister and niece had been diagnosed as VHL. Ultrasonography, CT, and MRI revealed bilateral adrenal tumors. Noradrenaline levels in serum and urine were elevated and 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, she had bilateral renal cysts and cerebellar hemangioblastoma. Bilateral adrenalectomies were performed and pathological diagnosis was pheochromocytoma. This is the seventh case of bilateral pheochromocytomas with VHL reported in Japan.