Posterior Reversible Encephalopathy Syndrome: The Spectrum of MR Imaging Patterns.

AIM: The aim of this study was to describe lesion patterns, distribution, and evolution in posterior reversible encephalopathy syndrome (PRES) in a larger single-center population. METHODS: Scans and follow-up, if available, of 50 patients with PRES between 2002 and 2011 were reviewed retrospectively. Lesion patterns, extent, and signal intensity changes were identified and graded on fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted images. Hemorrhagic changes were identified on T2* or susceptibility-weighted images, and gadolinium enhancement on T1-weighted images was identified if available. RESULTS: The most frequently affected regions on FLAIR were the frontal lobes in 54 %, occipital lobes in 34.3 %, and parietal lobes in 31.0 % of cases, thus 65.3 % in the posterior regions. Temporal lobes were affected in 10.6 %, the cerebellum in 6.5 %, and basal ganglia in 1.6 %. Division into vascular supply showed involvement in the anterior circulation in 66.5 % and in the posterior circulation in 33.5 % of cases. On diffusion-weighted imaging (DWI), vasogenic edema was observed in 6.9 %, cytotoxic edema in 9.1 %, and both in 2 % of cases. In 31.9 %, there was shine through, and in 15.9 %, there was shine through as well as cytotoxic or vasogenic edema. Topologic distribution on DWI showed affection of the frontal lobes in 43.5 %, occipital lobes in 25.8 %, parietal lobes in 17.7 %, temporal lobes in 11.3 %, and cerebellum in 1.6 %. T2* or susceptibility-weighted images showed spot-like hemosiderin accumulation in 17.2 % of cases. In 23.1 %, enhancement was seen. Follow-up magnetic resonance imaging showed complete resolution in 66.6 % of patients. CONCLUSION: The spectrum of imaging findings in PRES is wide. Almost always subcortical and cortical structures are involved. Although posterior changes are prominent in this syndrome, frontal involvement is more frequent than posterior on FLAIR imaging and DWI. On DWI, mixed patterns are not uncommon. Reversibility generally takes place independent of DWI pathology. Hypertension was not a prognostic factor.

[Facial and eye pain - Neurological differential diagnosis]. / Augenschmerzen aus neurologischer Sicht.

Head and facial pain are common in neurological practice and the pain often arises in the orbit or is referred into the eye. This is due to the autonomic innervation of the eye and orbit. There are acute and chronic pain syndromes. This review gives an overview of the differential diagnosis and treatment. Idiopathic headache syndromes, such as migraine and cluster headache are the most frequent and are often debilitating conditions. Trigemino-autonomic cephalalgias (SUNCT and SUNA) have to be taken into account, as well as trigeminal neuralgia. Trigemino-autonomic headache after eye operations can be puzzling and often responds well to triptans. Every new facial pain not fitting these categories must be considered symptomatic and a thorough investigation is mandatory including magnetic resonance imaging. Infiltrative and neoplastic conditions frequently lead to orbital pain. As a differential diagnosis Tolosa-Hunt syndrome and Raeder syndrome are inflammatory conditions sometimes mimicking neoplasms. Infections, such as herpes zoster ophthalmicus are extremely painful and require rapid therapy. It is important to consider carotid artery dissection as a cause for acute eye and neck pain in conjunction with Horner's syndrome and bear in mind that vascular oculomotor palsy is often painful. All of the above named conditions should be diagnosed by a neurologist with special experience in pain syndromes and many require an interdisciplinary approach.

Long survival of primary diffuse leptomeningeal gliomatosis following radiotherapy and temozolomide: case report and literature review.

OBJECTIVE: Primary diffuse leptomeningeal gliomatosis (PDLG) is a rare neoplasm with a short survival time of a few months. There is currently no standardized therapeutic approach for PDLG. - MATERIAL AND METHODS: We report on a 53-year-old male patient who presented with epileptic seizures, gait disturbance, paraparesis and sensory deficits in the dermatomes T8-10. - RESULTS: Magnetic resonance imaging (MRI) revealing numerous spinal and cranial gadolinium-enhancing nodules in the meninges and histopathology led us to diagnose primary diffuse leptomeningeal gliomatosis with WHO grade III astrocytic cells. Consecutively, the patient underwent craniospinal radiotherapy (30Gy) and 11 sequential cycles of temozolomide. This regimen led to partial tumor regression. Thirteen months later, spinal MRI revealed tumor progression. Second-line chemotherapy with 5 cycles of irinotecan and bevacizumab did not prevent further clinical deterioration. The patient died twenty-two months after diagnosis, being the longest survival time described thus far with respect to PDLG consisting of astrocytic tumor cells. - CONCLUSIONS: Radiochemotherapy including temozolomide, as established standard therapy for brain malignant astrocytomas, might be valid as a basic therapeutic strategy for this PDLG subtype.

Three cases of CLIPPERS: a serial clinical, laboratory and MRI follow-up study.

The aim of the study was to further determine the pathophysiology, clinical course, MRI-features and response to therapy of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), which has recently been proposed as a rare chronic inflammatory central nervous system disorder responsive to immunosuppressive therapy. Three patients with this rare entity underwent serial clinical and bimonthly MRI follow-up over a period of up to 16 months. Extensive laboratory work-up and brain biopsy were performed. Intravenous methylprednisolone or oral dexamethasone was administered as treatment, additionally cyclophosphamide in one patient. Clinically, diplopia, nystagmus, ataxia and facial paresthesia were the cardinal symptoms. Magnetic resonance imaging (MRI) disclosed patchy spot-like gadolinium enhancement in a "salt-and-pepper like appearance" in the pons, midbrain and cerebellum, in two cases with thalamic and in the other with spinal involvement. Brain biopsies demonstrated a predominantly angiocentric but also diffuse infiltration pattern by small mature lymphocytes. Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions. As discontinuation of steroids led to clinical relapse, one patient was treated with a further course of steroids and the other with steroids and cyclophosphamide as immunosuppressive therapy. This led to stable remission with only mild clinical residue and normalization of MRI. Extensive laboratory and radiological work-up could not identify any other cause of the disease. Of note, in two cases a marked elevation of IgE in serum was found initially and throughout the course. CLIPPERS seems to be a distinct inflammatory central nervous system disorder. It shows characteristic MRI core features. Extrapontine involvement seems to be frequent. Histologically it is characterised by predominantly angiocentric infiltration by small mature lymphocytes. A pathogenetic relationship between the elevated IgE levels and the perivascular infiltrates can be presumed. It is responsive to immunosuppressive therapy and can require prolonged or maintenance treatment.

Isolated degeneration of the posterior column as a distinct entity--a clinical and electrophysiologic follow-up study.

OBJECTIVE: The aim of the study was to better describe the long term clinical course and electrophysiologic and radiologic findings in isolated degeneration of the posterior column. METHODS: Four patients with the presenting symptoms of a progressive tabetic ataxia were followed up clinically and electrophysiologically over up to 15 years between 1997 and 2008. They received standardized neurological examinations, electrophysiologic testing with SEP, MEP, NCV, EMG, autonomic testing and cardiac evaluation, head and spine MRI, laboratory evaluation including CSF analysis. RESULTS: Progressive gait ataxia due to pallhypasthesia and loss of position sense with areflexia remained the only symptoms. Pes cavus deformity was a notable clinical feature in all cases. There was no involvement of other systems and all patients remained fully ambulatory. There was no cardiac involvement. Electrophysiology was characterized by absent cortical tibial SEP with normal lumbar complexes and normal nerve conduction studies and transcortical magnetic stimulation as well as sympathetic skin response. MRI of the cord was normal. Laboratory analysis and CSF were unrevealing. CONCLUSION: Isolated degeneration of the posterior column is a rare condition with a clinically benign course without progression involving other systems and characteristic electrophysiologic findings (isolated loss of cortical tibial-SEP with normal lumbar leads). Pes cavus deformity seems to be an unusual but typical clinical feature. The etiology is most likely a sporadic degenerative disease of the cord.

[Effects of MRI on the electrophysiology of the motor cortex: a TMS study]. / Auswirkungen der Magnetresonanztomografie auf die Elektrophysiologie des motorischen Kortex: eine Studie mit transkranieller Magnetstimulation.

PURPOSE: The increasing spread of high-field and ultra-high-field MRI scanners encouraged a new discussion on safety aspects of MRI examinations. Earlier studies report altered acoustically evoked potentials. This finding was not able to be confirmed in later studies. In the present study transcranial magnetic stimulation (TMS) was used to evaluate whether motor cortical excitability may be altered following MRI examination even at field strength of 1.5 T. MATERIALS AND METHODS: In 12 right-handed male volunteers individual thresholds for motor responses and then the length of the post-excitatory inhibition (silent period) were determined. Subsequently the volunteers were examined in the MRI scanner (Siemens Avanto, 1.5 T) for 63 minutes using gradient and spin echo sequences. MRI examination was immediately followed by another TMS session and a third 10 minutes later. As a control condition, the 12 subjects spent one hour in the scanner without examination and one hour on a couch without the presence of a scanner. RESULTS: After MRI examination, the silent period was significantly lengthened in all 12 subjects and then tended to the initial value after 10 min. Motor thresholds were significantly elevated and then normalized after 10 minutes. No significant effects were found in the control conditions. CONCLUSION: MRI examination leads to a transient effect on motor cortical excitability indicated by elongation of the post-excitatory inhibition and to an increase in motor thresholds in some subjects. These effects do not seem to be associated with a static magnetic field.

Substantia nigra hyperechogenicity and CSF dopamine depletion in HIV.

Dopaminergic dysfunction is thought to play a pivotal role in human immunodeficiency virus (HIV)-related dementia. Decreased dopamine (DA) levels in the cerebrospinal fluid (CSF) and neuronal loss in the substantia nigra (SN) have been reported in HIV-infected patients, suggesting nigrostriatal damage. Structural changes detectable as hyperechogenicity in transcranial ultrasound (TCS) scans of the SN have been reported in patients with Parkinson's disease (PD) and other neurological conditions. In this study, we assessed the echomorphology of the SN in 40 HIV-positive patients compared to 40 age- and sex-matched healthy controls and correlated these findings with CSF levels of DA and the metabolites homovanillic acid (HVA) and 3,4-dihydroxy phenylacetic acid (DOPAC) and with neuropsychologic performance. We observed that the SN of HIV-infected patients was hyperechogenic relative to that of controls (0.07 +/- 0.05 vs. 0.04 +/- 0.07 cm(2); mean +/- SEM; P < 0.001) and that this SN hyperechogenicity was correlated with decreased DA levels in the CSF, decreased CD4 cell count, and an impaired performance in the psychopathology assessment scale (AMDP) subtest for drive and psychomobility. An association to CDC stage, duration of HIV infection, or presence of HIV dementia was not observed. Our results indicate changes in the nigrostriatal system in HIV-infected patients that are detectable as hyperechogenic SN precede prominent extrapyramidal symptoms and cognitive dysfunction.

Endovascular treatment resolves non-hemorrhagic brainstem dysfunction due to tentorial dural AV fistula.

Tentorial dural arteriovenous fistulas (tDAVF) clinically present usually with subarachnoid and/or intraparenchymal hemorrhage. Reported rates range from 58% to 92% and neurological deficits occur in 79% to 92% of patients. This is due to venous congestion resulting from retrograde leptomeningeal venous drainage, which rarely, can be clinically silent. A 69-year-old woman presented with vertigo, double vision and gait instability. Cerebral digital subtraction angiography revealed a tDAVF with retrograde cerebellar venous drainage directed through the vein of Galen into the straight sinus. MRI showed extensive cerebellar edema due to venous congestion. Clinical manifestations of cerebellar and brainstem dysfunction resolved completely after transarterial embolization with N-butylcyanoacrylate.

Cerebral venous thrombosis after epidural blood patch: coincidence or causal relation? A case report and review of the literature.

We report on a female patient who developed post-dural puncture headache (PDPH) after epidural analgesia for delivery. Treatment with epidural blood patch led to complete headache remission and the patient was discharged. Two days later the patient was readmitted with hemihypaesthesia and mild hemiparesis of the right side. Magnetic resonance imaging showed a small left parietal cortical haemorrhage probably following cerebral venous thrombosis (CVT). Coagulation screening detected heterozygous Factor V mutation. Headache is a common symptom of PDPH and CVT. Review of the literature revealed five patients in puerperal state, who developed CVT in close temporal relationship after blood patch treatment for PDPH. Change of headache character with loss of postural influence was reported frequently before diagnosis of CVT was confirmed. These findings may indicate a causal relationship.

Evolution of purely infratentorial PML under HAART--negative outcome under rapid immune reconstitution.

Progressive multifocal leukoencephalopathy (PML) caused by the polyomavirus JC is a well-recognised complication of AIDS. Purely infratentorial manifestations are rare. Introduction of highly active antiretroviral therapy (HAART) has been associated with a reduction in morbidity and an improvement in overall survival among HIV-infected individuals. Recently, several reports have described adverse events in patients with PML who begin HAART and show evidence for immune reconstitution. We describe the clinical course of two patients with PML with purely infratentorial manifestation, whose clinical course deteriorated despite the successful introduction of HAART. Possible underlying immunological mechanisms are discussed.

Basal ganglia infarction mimicking glioblastoma.

Modern brain imaging techniques usually allow a very good differential diagnosis of intracerebral lesions, but in some cases the differential diagnosis is difficult. We report the case of a 52 year old male with acute brachiofacial paresis and a hyperintense lesion with mass effect and ring-enhancement in basal ganglia suspiciously to a tumor. The neurosurgeons recommend stereotactical brain biopsy for diagnosis, but the patient recovered in following time gradually and in repeated computer tomographic images contrast enhancement disappeared and a hypodense zone in the basal ganglia remains. Our case demonstrates that brain infarctions can mimick glioblastoma in taking cystic appearance and contrast enhancement. Stereotactic biopsy would have been a precipitated invasive procedure in this case.

Presumed pharmacologically induced change from upbeat- to downbeat nystagmus in a patient with Wernicke's encephalopathy.

A case of Wernicke's encephalopathy due to proven thiamine deficiency suffering from upbeat nystagmus (UN) changing to downbeat nystagmus (DN) after a latency of 1 year is presented. The case was also notable for the finding of positive oligoclonal bands in the CSF. The underlying pathophysiological mechanisms and a possible effect of baclofen treatment are discussed.

[Restenosis of carotid artery after stenting associated bleeding in the vascular wall]. / Karotis-Restenose durch Stent-assoziierte Einblutung in einen atheromatös-fibrösen Herd.

Stenting is an alternative procedure to the endarterectomy in patients with severe (> 70 %) symptomatic stenosis of the carotid artery. Its value is actually tested in large controlled randomised multicenter studies. Many vascular surgeons postulate no benefits for the patients but a higher complication rate for the stenting procedure. Especially the possibility of mechanical irritations of the endothel caused by the stent itself seems to be a major problem. Despite thousands of stenting procedures of the carotid artery, there exist no reports of patients with direct mechanical irritation of the carotid artery through the stent itself. It is only published a higher risk of embolisation. This case of a 71-years old patient, who had a irradiation of the head and neck region 5 years ago for cancer therapy, is presented here. After the irradiation (60 gr) the stent was not so fixed in the carotid wall as usually. Therefore the stent caused a mechanical irritation of the sclerotic plaque with a bleeding in the carotid wall and subsequent severe stenosis at the end of the stent with higher level of infarction volume. This case demonstrates the risk of the theoretical postulated irritation of the endothel through the stent itself in a high movement region.