Progression of chronic subdural haematomas in an infant boy after abusive head trauma.

Abusive head trauma is a serious form of child abuse that can lead to severe neuropsychological sequelae or death in infants. In questionable cases, without a confession from the caregivers and ambiguous clinical information, evidence for the diagnosis of abusive head trauma is often based on typical patterns that have been observed in neuro-imaging. This study shows the progressive evolution of multifocal chronic subdural haematomas, including re-bleedings, in a case of abusive head trauma in an infant boy who was documented with repeated magnetic resonance imaging. The chronic subdural haematomas occurred during closely monitored in-patient rehabilitative care, and repeated maltreatment did not appear to be likely. Due to excessive growth, neurosurgical intervention with endoscopic craniotomy, evacuation of the subdural haematomas and temporal external cerebrospinal fluid drainage was performed with a favourable recovery. This study discusses the current pathophysiological knowledge concerning the development and clinical course of chronic subdural haematomas and draws relevant conclusions for the clinical practice and psychosocial management of caring for victims of abusive head trauma.

Cerebral hemiatrophy associated with hippocampal sclerosis following a single prolonged febrile seizure.

The etiological relation of prolonged febrile seizures with hippocampal sclerosis and cerebral hemiatrophy is controversial. Causal relationship is mainly adopted from retrospective statistical analysis and data from epilepsy surgery. We report a 17-month-old boy who had a prolonged febrile seizure with a transient postictal flaccid hemiparesis and anisocoria. Family history was unremarkable. Magnetic resonance imaging (MRI) revealed abnormal results in the right hippocampal area where diffusion-weighted sequences showed increased signal intensity consistent with acute neuronal edema. Repeat MRI 5 months later demonstrated sclerosis and atrophy of the right hippocampus in association with an increased T2-weighted signal and atrophy of the right frontal, temporal, and parietal lobe. In addition, 18-fluorodeoxyglucose positron emission tomography and 99mTc-ECD single-photon emission computed tomography revealed glucose hypometabolism and decreased perfusion in the right hemisphere, respectively. A final MRI, 12 months following the seizure, was widely unchanged. Interestingly, during a follow-up of 42 weeks, only minor motor deficits were observed. This case uniquely presents the acute onset of hippocampal sclerosis and, consecutively, cerebral hemiatrophy after a single febrile seizure. This suggests that a single prolonged febrile seizure may cause global morphological changes of the brain, not only affecting hippocampal formation.

MRI of the eye muscles in a case of ophthalmoplegia caused by common carotid artery occlusion suggests ischemic myopathy.

Ocular muscle palsies following carotid artery disease is thought to be caused by ischemia of the cranial oculomotor nerves but it may also be due to ischemia of the extraocular muscles (EOM). We studied a patient with common carotid artery occlusion syndrome (CCAOS) to elucidate the two competing hypotheses. MRI and sonography of the orbita showed oedematous swelling of all left EOM. MRI short-tau inversion recovery (STIR) sequences showed hyperintensities and a prolonged T2-relaxation time in EOM indicating muscle oedema. It decreased within two weeks as ophthalmoplegia improved. For several reasons ischemic EOM myopathy rather than ischemic neuropathy seems to be the morphological correlate of ophthalmoplegia after ipsilateral CCAOS in this patient.

Cranial pachymeningitis: a rare neurological syndrome with heterogeneous aetiology.

Cranial pachymeningitis is a poorly understood syndrome, defined by leptomeningeal thickening and typical gadolinium enhanced MRI. The heterogeneous clinical and aetiological features of five patients with both focal and diffuse pachymeningitis are presented. The initial symptoms included headache (n=3), sensory Jackson seizures (n=1), hemiparesis (n=1), episodes of short lasting hemiataxia (n=1), hemihypaesthesia (n=1), aphasia (n=1) and confusion (n=2). MRI scans revealed focal (n=3) or diffuse (n=2) leptomeningeal gadolinium enhancement and cortical swelling (n=4). In addition, one case presented with a subarachnoid and a second with an intracerebral haemorraghe. CSF findings were variable and showed clear lymphomonocytic pleocytosis in 3/5 cases. Infectious diseases were extensively excluded in all cases. Leptomeningeal biopsies of two cases revealed perivascular inflammation, indicating central nervous system vasculitis. In the cases presented, pachymeningitis was caused by primary central nervous system vasculitis (n=2) and rheumatoid arthritis (n=2). In one case, the cause remained unclear.

Bedside screening for executive dysfunction in patients with subcortical ischemic vascular disease.

OBJECTIVE: We investigated several executive bedside tests for their effectiveness in the routine clinical diagnostics of dysexecutive syndrome in subcortical ischemic vascular disease (SIVD). METHODS: Five executive tests, CLOX, the Tower of London (ToL), a cognitive estimation test (CET), a verbal fluency test, and the Five-Point Test, were examined in 17 patients with marked cerebral microangiopathy in cranial MRI and clinical symptoms of SIVD. The test accuracy for discriminating the patients from 17 healthy comparison subjects closely matched for age, gender and level of education was determined. RESULTS: Aside from the CET we found a significant lower performance of the patients with SIVD in four of the five used executive tests. In receiver operating characteristic (ROC) analyses the accuracy of CLOX 1 showed excellent results for distinguishing between patients and comparison subjects (area under the curve (AUC) 0.901), while the ToL (AUC up to 0.845) and the productivity in the phonemic verbal fluency test (AUC 0.829) achieved a good accuracy. Differently the accuracy of the figural fluency was only poor to fair (AUC 0.706). However, the Youden Indices of the significant executive variables showed a wide range from 0.25 to 0.82. CONCLUSIONS: Based on our data we consider CLOX, the ToL and the verbal fluency test promising executive bedside test concepts for diagnosing the dysexecutive syndrome in SIVD in clinical routine. Particularly for CLOX and the ToL a further psychometric evaluation is required.

Japanese encephalitis in Western Europe.

Japanese encephalitis is the leading cause of viral encephalitis in Asia. In Western Europe, however, Japanese encephalitis has not been reported so far. We report on a 59-year-old German woman with typical neurological symptoms and clinical presentation 8 days after a journey to northern China. The case report summarizes the patient's clinical course as well as pathological findings in blood samples, the cerebro-spinal fluid, MRI scans, and EEGs.

Structural findings in the basal ganglia in genetically determined and idiopathic Parkinson's disease.

A bilateral compensatory increase of basal ganglia (BG) gray matter value (GMV) was recently demonstrated in asymptomatic Parkin mutation carriers, who likely have an increased risk to develop Parkinson's disease (PD). We hypothesized BG morphological changes in symptomatic Parkin mutation carriers (sPARKIN-MC) and idiopathic PD patients (iPD) after the occurrence of PD symptoms, reflecting the breakdown of compensatory mechanisms. Nine sPARKIN-MC, 14 iPD, and 24 controls were studied clinically and with voxel-based morphometry. Analysis of variance revealed mainly BG decrease of GMV in sPARKIN-MC and to a lesser extent in iPD. However, a slight increase in GMV was also found in the right globus pallidus externus in sPARKIN-MC and in the right putamen in iPD. This may reflect a structural correlate of functional compensation that can only partially be maintained when nigrostriatal neurodegeneration becomes manifest. Simple regression analyses with the UPDRS-III and disease duration score revealed a distinct more bilateral linear decrease of BG GMV in sPARKIN-MC than in iPD that may correspond to previous findings showing a symmetric reduction in putaminal (18)F-DOPA-uptake and bilateral manifestation of symptoms in sPARKIN-MC. In symptomatic PD, BG are subject to a progressive atrophy, which gradually increases with disease severity and duration.

Preoperative thrombocytosis predicts poor survival in patients with glioblastoma.

Thrombocytosis, which is defined as a platelet count greater than 400 platelets/nl, has been found to be an independent predictor of shorter survival in various tumors. Release of growth factors from tumors has been proposed to increase platelet counts. Preoperative platelet counts and other clinical and hematological parameters were reviewed from the records of 153 patients diagnosed between 1999 and 2004 with histologically confirmed glioblastoma in order to evaluate the prognostic significance of preoperative thrombocytosis in these patients. The relationship between thrombocytosis and survival was initially analyzed in all patients regardless of further therapy. Univariate log-rank tests showed that the median survival time of 29 patients with preoperative thrombocytosis (19%) was significantly shorter (4 months; 95% confidence interval [95% CI], 3-6 months) compared to 124 patients with normal platelet counts (11 months; 95% CI, 8-13 months; p = 0.0006). Multivariate analysis (Cox proportional hazards model) confirmed preoperative platelet count, age, prothrombin time, and activated partial thromboplastin time to be prognostic factors of survival (all p < 0.05). In a subset of patients (only operated patients with radiation therapy with or without additional chemotherapy), survival was likewise significantly shorter when preoperative thrombocytosis was diagnosed (6 months; 95% CI, 4-12 months) compared to patients with normal platelet count (13 months; 95% CI, 11-15 months; p = 0.0359). In multivariate analysis, age, platelet count, preoperative prothrombin time, and degree of tumor resection retained significance as prognostic factors of survival (all p < 0.05). The results of our study demonstrate preoperative thrombocytosis to be a prognostic factor associated with shorter survival time in patients with glioblastoma.

Lumbosacral glioblastoma and leptomeningeal gliomatosis complicating the course of a cervicothoracic astrocytoma WHO grade II.

CASE REPORT: The reported female patient underwent sub-total resection of an intra-medullary cervicothoracic astrocytoma classified as WHO grade II in 1984 at the age of 18 months and received local irradiation. In 1989, a local recurrence was diagnosed and a partial resection was performed. Sixteen years later, a small recurrent cervicothoracic tumour was found and spinal seeding to the equine nerve roots and the left cerebellar cortex was apparent on MRI. The patient was implanted with a ventriculoperitoneal shunt for a pseudo-tumour cerebri producing papilloedema, which eventually lead to amaurosis. After an extended biopsy, the invasive lumbosacral tumour was classified as glioblastoma multiforme. Two months later, the patient died after rapid progression of the caudal cranial nerve dysfunction. DISCUSSION AND CONCLUSION: Anaplastic progression and dissemination of spinal astrocytomas even two decades after initial diagnosis and treatment are rare. Therapies and diagnostic follow-up strategies are discussed.

Recurrent seizures do not cause hippocampal damage.

Neuronal consequences of recurrent single epileptic seizures have been discussed controversially for some time. Various cross-sectional magnetic resonance imaging (MRI) studies have shown a positive correlation between the severity of epilepsy and the extent of hippocampal damage. However, the open question whether recurrent epileptic seizures induce hippocampal structural pathology can be assessed only in longitudinal studies. The few recent follow-up studies have revealed conflicting results. In the current MRI study we have employed volumetry and T2 relaxometry to quantify hippocampal structural changes of patients with chronic partial epilepsies over a period of 3 years. Our main findings demonstrate that these patients who experience continuing epileptic seizures do no show any development of new pathology or any relevant deterioration of pre-existing hippocampal structural lesions. This argues against the assumption that recurrent epileptic seizures cause or increase structural hippocampal damage.

Dural arteriovenous fistula of the lumbar spine presenting with subarachnoid hemorrhage. Case report and review of the literature.

The authors report on a patient presenting with subarachnoid hemorrhage (SAH) that was initially attributed to an aneurysm of the right internal carotid artery. During surgical exploration and placement of a clip, however, it was observed that the aneurysm had not ruptured. Diagnostic workup including spinal magnetic resonance imaging revealed a vascular malformation of the lumbar spinal canal within a subarachnoid hematoma. Spinal angiography demonstrated a spinal dural arteriovenous fistula (DAVF) (Type I spinal arteriovenous malformation) with a feeding vessel arising from the L-4 radicular artery. In the literature, SAH due to spinal DAVFs is rare; only cases of dural fistulas of the craniocervical junction and the cervical spine have been reported. This is the first case of SAH that can be attributed to a lumbar DAVF. Although unusual even in cases of cervical DAVF, SAH as a presenting symptom may occur in spinal DAVF of any location. Nontraumatic SAH should not be prematurely attributed to the rupture of an intracranial aneurysm if the clinical findings and imaging results are inconclusive.

Multiphasic disseminated encephalomyelitis associated with streptococcal infection.

Multiphasic disseminated encephalomyelitis (MDEM) is a rare peculiar diagnosis which is defined as acute demyelinating central nervous system disease with relapses occurring only within 4 weeks of initial manifestation. This report describes the case of a 6-year-old boy with MDEM diagnosed by clinical findings and magnetic resonance imaging. The disease had a biphasic evolution, and with a second course of high-dose corticosteroids a complete recovery without further relapse was obtained during the following 18 months. Serological evidence of streptococcal infection as specific trigger for MDEM was given. Thus this report raises the question whether an additional penicillin prophylaxis could be valuable for prevention of streptococcus-associated MDEM relapses.

Isolated unilateral cortical oedema and complex partial seizures in association with coxsackievirus B infection.

A unilateral cortical oedema in association with coxsackievirus B infection is reported. A 10-year-old girl presented with right hemiparesis and complex partial seizures. The cerebral MRI showed a unique pattern of isolated unilateral cortical oedema sparing the white matter with intravascular gadolinium enhancement of the left hemispheric sulcal veins. With anticonvulsant medication, the patient recovered within two weeks and MRI abnormalities were completely resolved after four weeks, whereas the EEG left hemispheric slowing showed delayed normalisation over the following five months.