Laminar Necrosis Research Articles

Posterior reversible encephalopathy syndrome in children: Radiological and clinical findings – A retrospective analysis of a German tertiary care center

PurposeTo report the radiological and clinical spectrum of posterior reversible encephalopathy syndrome (PRES) in children in a German tertiary referral center. MethodsThe radiological report data bases of the authors' university hospitals were searched for paediatric patients with PRES. Clinical and paraclinical data as well as various imaging features at symptom onset and during follow-up were tabulated in patients fulfilling the criteria for PRES. ResultsA total of 18 paediatric patients with PRES were included into the study. Mean age was 9 years (IQR 7–12), 38.9% were females. Most frequent predisposing causes were renal and haemato-oncologic diseases frequently associated with endotheliotoxic cytostatic medication. Frontal lesions occurred as frequently as parietal lesions followed by occipital lesions. The superior frontal sulcus topographic lesion pattern occurred as frequent as the parieto-occipital one. In 38% of cases residual lesions were encountered with focal laminar necroses being most frequent. Initial clinical syndromes associated with PRES included seizures in 18, altered mental state in 5, and hemiparesis and visual disturbances in 2 children. Mean arterial blood pressure at onset of PRES was 140/85 mmHg (IQR systolic: 124–169, diastolic: 78–93 mmHg). ConclusionPaediatric PRES in this cohort comprises a broad radiological and clinical spectrum. The occurrence of frontal lesions, a superior frontal sulcus associated lesion pattern, and the development of focal laminar necrosis appear to be frequent in children.

A patient with demyelination, laminar cortical necrosis, and rhabdomyolysis associated with hypernatremia

A 60-year-old man with renal failure and intraabdominal abscess formation probably due to perforation of the colon underwent laparotomy on the sixth hospital day. He developed respiratory infection, deterioration of renal failure, and heart failure resulting in severe respiratory insufficiency after laparotomy. He was placed on mechanical ventilation using sedatives and muscle relaxant and was treated with antibiotics, steroids, and a diuretic. The value of serum sodium jumped from 146 to 164 mEq/L in 2 days. Sodium infusion was discontinued, and hypernatremia decreased. He fell into a coma and demonstrated generalized convulsions after mechanical ventilation was discontinued. His head computed tomography did not indicate any pathologic findings, and his convulsions were not controlled so that he was again placed on mechanical ventilation. The laboratory findings revealed rhabdomyolysis (18936 IU/L) 5 days after the normalization of hypernatremia. Mechanical ventilation and hemodialysis were terminated after the convulsions were controlled and the renal failure improved on the 82nd hospital day. Head magnetic resonance imaging exhibited that multiple hyperintensity lesions in the white matter with linear signal changes in both occipital cortex. He remained unconscious for 6 months. This is the first case that demonstrated demyelination, laminar cortical necrosis, and rhabdomyolysis associated with hypernatremia. Rhabdomyolysis after rapid occurrence of hypernatremia might be a laboratory sign of concomitant demyelination.

Early Histological Changes in the Porcine Aortic Media After Thoracic Stent-Graft Implantation

To describe the histological findings in the aortic wall 5 days after thoracic endovascular aortic repair (TEVAR) in a porcine model. Two overlapping stent-grafts were implanted in each of 6 juvenile pigs, covering the entire descending thoracic aorta (DTA). On the 5(th) postoperative day, tissue samples were taken from the DTA in each animal. Medial thickness and medial necrosis were quantified and compared to measurements from the aortas of 6 control animals. Significant medial thinning was observed in stent-covered regions in the test animals. At the proximal landing zone, aortic wall thickness changed from 1387±68 to 782±74 µm within the covered aortic segment (p = 0.028); at the distal landing site, the wall thickness was 365±67 µm within the stent and 501±57 µm distally (p = 0.028). In the overlap zone, the aortic wall measured 524±122 vs. 1053±77 µm in native controls (p = 0.004). Aortic thickness proximal to the graft did not differ from the proximal region of native aortas (1468±96 vs. 1513±80 µm, p = 0.423), but the aorta was significantly thinner distal to the stent (707±38 vs. 815±52 µm, p = 0.004). Laminar necrosis constituted 38%±7% of the media in the proximal landing zone, 54%±4% in the overlap zone, and 46%±13% in the distal landing zone. In this porcine model, significant medial thinning and necrosis of the stented aorta was observed. The findings suggest an early phase of vulnerability of the aortic wall, before scarring and adaptive changes have strengthened the residual aorta.

Heroin inhalation-induced unilateral complete hippocampal stroke

A 33-year-old man presented to our clinic with amnesia 48 hours after his first heroin inhalation. Examination showed lateral tongue biting and anterograde amnesia demonstrated by impaired performance on verbal and visual Wechsler Memory Scale–Revised tests carried out 10 days after onset, suggesting hippocampal involvement. Magnetic resonance imaging (MRI) of the brain was performed 48 hours after heroin snorting and evoked cortical laminar necrosis (CLN) of the left hippocampus without vascular abnormality. This is the first description of complete hippocampal CLN as a complication subsequent to acute intranasal heroine abuse. While the pathogenic mechanism remains uncertain, our case provides a very specific MRI lesion pattern and highlights the risk of intranasal heroin uptake-induced neurological complication.

Discrete progression of migraine-induced focal cerebral infarction

Migraine-induced infarction is rare and possibly overdiagnosed [1]. Although the relationship between migraine and stroke has historically been a subject of controversy, recent evidence has lent support to the notion that migraine and stroke are co-morbid conditions linked by complex pathophysiological processes [2]. Migraine with aura is more clearly associated with increased stroke risk than is common migraine, with studies implicating smoking [3] and elevated plasma homocysteine [4], among a host of factors, as mediating risk. There appears to be a predilection for stroke localization within the posterior cerebral artery (PCA) territory [5]. The patient reported herein represents an example of migraine-induced infarction. A 66-year-old man with a history adolescent-onset classic migraine presented with prolongation of his typical symptoms, consisting of scintillating scotoma in the right visual field, followed by perioral parethesia, mild dysarthria, and anomic aphasia, and concluding with relatively mild occipital headache. Typical frequency was one to four episodes monthly with a duration of 3–4 h each. In contrast, the duration of the presenting episode was protracted, lasting between 48 and 72 h. Symptoms responded briskly to solumedrol 125 mg IV and subcutaneous sumatriptan 6 mg. The patient reported one other life-time episode suggesting migraine status several years previously with no residual symptoms. The neurological examination was unremarkable; subjectively, however, the patient reported persisting visual scotoma located 10 –15 to the right of foveation. Since the event, he also complained of increased forgetfulness requiring the use of compensatory lists and which contributed to his decision to delegate responsibility for managing the family finances to his spouse. Neuropsychological evaluation revealed deficits in memory for verbal material and confrontation naming; his MMSE score was 27/30. MRI with contrast revealed a focal gyral pattern of signal hyperintensity on T1-, T2-weighted, and FLAIR sequences, with associated heterogeneous enhancement consistent with laminar necrosis from evolving subacute to chronic infarction in the left PCA distribution, affecting the mesial occipital region. Scattered, punctate areas of T2 and FLAIR hyperintensity were also noted in subcortical white matter (Fig. 1). Mass lesion, vascular malformation, and focal cerebritis were excluded. EEG obtained 2 years prior to that event was normal, effectively ruling out seizure disorder. Carotid Doppler study showed mild stenosis (16–49 %) of the internal carotid arteries bilaterally. Pertinent laboratory studies including ESR (10) and CRP (0.411) were normal, but homocysteine was elevated (15.1). TSH, vitamin B12, folate, and RPR were unremarkable. Migraine status with characteristic aura recurred 21 months later. Neurological examination following that event was remarkable only for a homonymous right superior quandrantanopia. Follow-up neuropsychological evaluation showed interval decline in memory, speed of visual search, cognitive flexibility, problem solving, and S. P. Cercy (&) Department of Psychiatry, New York University School of Medicine, 423 E 23rd Street, New York, NY 10010, USA e-mail: Steven.Cercy@va.gov

Pathophysiology of Stroke-Like Episodes in MELAS: A Possible Role of Neuron-Astrocyte Uncoupling in Cellular Damage during Synaptic Activation under Primary Oxidative Phosphorylation Defect (P01.033)

Objective: To speculate the pathophysiological mechanism of stroke-like episodes (SEs) in MELAS based on clinical data and current concept of neuron-astrocyte communications. Background The pathogenesis of SEs remains unknown. We previously reported that SEs are non-ischemic neurovascular events presumably associated with neuronal hyperexcitability (Neurology 2002, Curr Neurovasc Res 2005, Future Neurology 2010), however, the basic molecular mechanism underlying neuronal hyperexcitability remains unknown. It has been demonstrated that astrocytic processes sense synaptic activity and transmit the signals to the arterioles. Astrocytes communicate with a number of neurons or astrocytes by releasing gliotransmitters. Thus, gliotransmitters are considered to control synaptic transmission, neuronal synchrony and neuronal excitability. During synaptic activation, neurons preferentially use lactate which is continuously supplied through astrocyte-neuron lactate shuttle. Design/Methods: Retrospective review of 30 consecutive SEs in 8 patients with MELAS (A3243G (n=7), G13513A (n=1)) who were admitted to our hospital since 1990. Results: Common presenting symptoms included headache, visual symptoms, altered levels of consciousness or seizure, and psychiatric symptoms. Seizure developed during the course of SEs (69%), suggesting sustained neuronal hyperexcitability. SEs were often accompanied by focal hyperperfusion, focal periodic epileptiform discharges, spreading cortical edema and cortical laminar necrosis. Focal lactate accumulation with leptomeningeal vasodilatation was seen at early stage. Calcitonin gene-related peptide (CGRP) was elevated in CSF. Conclusions: SEs are episodic events characterized by neuronal hyperexcitability, increased anaerobic glycolysis, vasogenic edema and vasodilatation. We speculate that 1) sustained astrocyte activation following synaptic activation contributes to focal periodic epileptiform discharges and hyperperfusion, 2) trigeminovascular activation causes headache, 3) limited availability of ATP impairs sodium-dependent glutamate transporter, resulting in decreased clearance of glutamate from the synaptic cleft, 4) low extracellular adenosine level due to decreased ATP production may cause disinhibition of synaptic activity, and 5) sustained release of lactate from activated astrocytes potentially abort glycolytic pathway. Neuron-astrocyte uncoupling may contribute to cellular damage during SEs. Disclosure: Dr. Iizuka has nothing to disclose. Dr. Hamada has nothing to disclose. Dr. Sakai has nothing to disclose.

Cerebral Air Embolism with Upper Endoscopy: Two Patients, Three Events (P02.188)

Objective: Upper endoscopy is a frequent procedure with uncommon but known risks of aspiration, perforation and hemorrhage. Less well known are cerebral complications due to endoscopy. Background Upper endoscopy (UE) carries a rare risk of cerebral air embolism. Previously reported cases are isolated. Most involve the right hemisphere, probably because of patient positioning. We report two cases of cerebral air embolism with UE, one case unique because symptoms recurred and imaging studies demonstrated bilateral emboli. Design/Methods: Case Reports. Results: A 55 year old with a history of esophageal cancer required UE with dilation procedures for dysphagia. During a procedure he had an episode of left hemiparesis which resolved. Cerebral magnetic resonance imaging at that time showed bilateral abnormalities; the patient had no permanent deficit. 27 months later during another endoscopy he developed dense left hemiparesis with hypotension. Computerized tomography (CT) demonstrated a pattern of intravascular air over the right hemisphere. Despite urgent hyperbaric therapy, there was massive infarction. With aggressive medical therapy he avoided hemicraniectomy and survived with a left hemiparesis. An independent 91 year old with biliary obstruction had endoscopic retrograde cholangiopancreatography and developed hypotension with seizures. CT showed intracerebral air embolus. Magnetic resonance imaging showed cortical ischemia consistent with laminar necrosis. She expired, primarily as a consequence of global hypoxia. Conclusions: Air embolus is a risk of UE. Acute neurologic change during UE should raise suspicion for cerebral air embolus. Cardiac arrythmia or arrest due to air embolus can cause hypoxic-ischemic encephalopathy. Hyperbaric therapy has been reported to benefit cases of cerebral air embolus. Emboli or symptoms related to cerebral air embolus from upper endoscopy can be transient, recurrent and bilateral. Disclosure: Dr. Taylor has nothing to disclose. Dr. Benton has nothing to disclose. Dr. Schneider has nothing to disclose.

Utility of Diagnosing Various Histological Patterns of Diffuse Chronic Hypoxic Placental Injury

To examine the clinicopathologic correlations of three histological patterns of diffuse chronic hypoxic placental injury (preuterine [PR], uterine [UH], and postuterine [PU]), a retrospective statistical analysis of a large 14-year placental database was performed. Of 5097 placentas between 20 and 43 weeks of gestation examined consecutively, 4413 did not feature histological chronic placental hypoxia, while 684 did. In the latter, maternal hypertensive disorders, diabetes mellitus, abnormal cardiotocography and Dopplers, cesarean sections, inductions of labor, and fetal growth restriction, as well as other placental hypoxic lesions and decidual arteriolopathy, were statistically significantly more common than in the remaining placental material. Two hundred eighty-nine PR cases featured the most advanced gestational age and meconium staining; 237 UH cases featured severe preeclampsia, decidual arteriolopathy, villous infarction, membrane laminar necrosis, microscopic chorionic pseudocysts, excessive extravillous trophoblasts, and maternal floor multinucleate trophoblastic giant cells; and 158 PU cases featured the lowest placental weight and the highest prevalence of abnormal Dopplers, umbilical cord compromise, fetal growth restriction, cesarean section rate, and complicated 3rd stage of labor. The specificity of chronic hypoxic patterns of placental injury was much higher than the sensitivity, with the highest specificity for an excessive amount of extravillous trophoblasts. Diagnosing various hypoxic patterns of placental injury by histology may help to clarify the etiopathogenesis of a significant proportion of complications of pregnancy and abnormal fetal or neonatal outcomes. The patterns should help to retrospectively diagnose placental hypoxia, even in clinically unsuspected cases.

MRI Finding in Delayed Extensive Brain Lesions after Radiation Therapy :Cortical Laminar Necrosis and White Matter Myelinolysis

The focal and diffuse cerebral white matter injury can be caused by treatment with radiation therapy for cranial tumors. However, the literature rarely describes a MRI finding in radiation-induced delayed extensive cerebral injury. Our objective was to report a rare case who had a delayed extensive hyperintensity injury in brain on MRI after radiation therapy due to nasopharyngeal cancer. A MRI was performed on a 40-year-old patient with extensive brain damage who had the radiation therapy two years ago.MRI finding was evaluated. On MRI, T2-weighted MRI showed an extensive hyperintensity after treated by irradiation. The radiographic pattern of extensive cerebral injury is relatively distinct. It involves the white matter and gray matter in cerebral, cerebellum, medulla oblongata, pons, internal capsule and thalamus bilaterally. Our observations demonstrate that the extensive hyperintensity lesions in brain on MRI after radiation therapy is a cortical laminar necrosis and white matter myelinolysis.

Serial EEG and MRI changes in status epilepticus‐induced excitotoxic neuronal necrosis

Prolonged status epilepticus may directly cause selective neuronal necrosis due to excitotoxic mechanisms, as observed in experimental models and described in case reports. A 36-year-old woman presented with right hemiplegia and aphasia following a generalised tonic-clonic status epilepticus of two hours duration. Accompanying serial MRI with advanced imaging techniques, EEG and histopathology of the cortical tissue of the patient were all compatible with excitotoxic neuronal necrosis. In this histopathologically-proven rare case of status epilepticus-induced excitotoxic neuronal injury, the observation of delayed cortical laminar necrosis on MRI, together with paroxysmal lateralised epileptiform discharges on the EEG, suggests that these changes may be an early sign of impending and ongoing excitotoxic neuronal injury and delayed cell death caused by glutamate release due to excessive neuronal firing in status epilepticus.

頭部MRI上vascular boundary zoneにcortical laminar necrosisを呈した熱射病の1例

頭部MRI上vascular boundary zoneにcortical laminar necrosisを呈した熱射病の1例

The clinical and radiological spectrum of posterior reversible encephalopathy syndrome: the retrospective Berlin PRES study

The aim of the study was to characterize the clinical and radiological spectrum of posterior reversible encephalopathy syndrome (PRES) in a large cohort. The radiological report data bases of the authors' university hospitals were searched for patients with PRES. Various imaging features at onset of symptoms and on follow-up as well as clinical and paraclinical data were tabulated in those patients fulfilling the criteria for PRES. Exploratory univariate analyses were performed. A total of 96 patients with PRES were included into the study. Wide differences in lesion location, diffusivity, distribution pattern, edema severity, hemorrhage, underlying diseases, symptoms, mean arterial pressure (MAP) and coagulation status were encountered. Hemorrhage occurred significantly more frequently in patients with altered coagulation state and was significantly associated with higher edema grades and with the presence of cytotoxic edema. There was a significant difference in MAP between toxic associations with higher MAP in infection, eclampsy and autoimmune disorders, while lower MAP was found in chemotherapy and immunsupression. In 82% of patients complete or near complete resolution of edema was noted during follow-up. Higher MAP levels were associated with incomplete edema resolution. In 43% of patients residual lesions were seen with a relatively even distribution between focal gliosis, infarction, posthemorrhagic residua, atrophy and laminar necrosis. PRES in this large hospital-based retrospective study comprises a wide radiological and clinical spectrum. Residual lesions were encountered more frequently than commonly expected. Our results point towards a differential contribution of high blood pressure to the course of PRES in different underlying etiologies.

Typical CT and MRI features of cortical laminar necrosis

A 47-year-old woman with a prior medical history of SLE and chronic arterial hypertension developed a post-anoxic coma after a prolonged cardiac arrest. She also presented a convulsive status epilepticus.

Extrapontine Myelinolysis and Cortical Laminar Necrosis Caused by Severe Hypernatremia following Peritoneal Lavage for Ruptured Hydatid Cyst of the Liver

Extrapontine myelinolysis (EPM) and cortical laminar necrosis (CLN) have rarely been reported in association with severe hypernatremia. We describe a patient with EPM associated with CLN following severe hypernatremia due to hypertonic peritoneal lavage after a ruptured hydatid cyst of the liver. Clinical and neuroimaging findings in acute stage and serial brain MRI at two and five month follow-up are discussed in detail.

Patterns of accentuated grey–white differentiation on diffusion-weighted imaging or the apparent diffusion coefficient maps in comatose survivors after global brain injury

To determine what disease entities show accentuated grey-white differentiation of the cerebral hemisphere on diffusion-weighted images (DWI) or apparent diffusion coefficient (ADC) maps, and whether there is a correlation between the different patterns and the cause of the brain injury. The DWI and ADC maps of 19 patients with global brain injury were reviewed and evaluated to investigate whether there was a correlation between the different patterns seen on the DWI and ADC maps and the cause of global brain injury. The ADC values were measured for quantitative analysis. There were three different patterns of ADC decrease: a predominant ADC decrease in only the cerebral cortex (n=8; pattern I); an ADC decrease in both the cerebral cortex and white matter (WM) and a predominant decrease in the WM (n=9; pattern II); and a predominant ADC decrease in only the WM (n=3; pattern III). Pattern I is cerebral cortical injury, suggesting cortical laminar necrosis in hypoxic brain injury. Pattern II is cerebral cortical and WM injury, frequently seen in brain death, while pattern 3 is mainly WM injury, especially found in hypoglycaemic brain injury. It is likely that pattern I is decorticate injury and pattern II is decerebrate injury in hypoxic ischaemic encephalopathy.Patterns I and II are found in severe hypoxic brain injury, and pattern II is frequently shown in brain death, whereas pattern III was found in severe hypoglycaemic injury.

Cerebellar hematoma in a carrier of the A3243G MELAS mutation

Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is often related to the A3243G mutation of the mitochondrial DNA, accounting for about 80% of typical cases, with a relevant phenotypic variability. Hemorrhagic strokes are quite unusual in these patients. A case of recurrent brain hematomas has been reported in a MELAS patient carrying the G13513A mutation, with clinical onset at age 31 [1]. Another fatal case of intracerebral hemorrhage was reported in a MERRF/MELAS overlap syndrome due to a T8356C mutation [2]. Finally, a putaminal hemorrhagic stroke was described in a MERRF patient carrying the A8344G mutation, with clinical onset at age 26 [3]. Here, we report the case of a 37-year-old male arriving to our attention because of a two-week history of headache associated with nausea, vomiting, and dizziness. A brain MRI scan showed a left hemispheric cerebellar hemorrhage at a stage compatible with the onset of the symptoms. The patient neither had history of arterial hypertension, any hemorrhagic risk-factor, diabetes, nor was taking any medication. A few years before, he was found to be asymptomatic carrier of the A3243G mitochondrial DNA mutation (heteroplasmy confirmed on a blood sample), since a MELAS diagnosis was made on his younger brother expressing a typical phenotype. On current examination, our patient presented slight left ataxic hemiparesis and hypoesthesia. A cerebral angiography failed to show any vascular abnormality. Since our patient was carrier of a MELAS mutation, we decided to assess its putative clinical burden. An EEG showed generalized electrogenetic dysfunction with a single generalized epileptic spike discharge; EMG suggested slight myopathic signs; mild perceptive bilateral deafness was also present. A venous lactic acid test with compression of the right arm was significant (baseline values 2.9 mmol/L; increase up to 7.5 mmol/L after 3 min; NV 0.44–2.22 mmol/L). No hearth conduction defects were present. A week later, on a control CT scan, progressive blood reabsorption was noted and basal ganglia calcifications were not observed; the headache had improved and the neurological examination was normal. Iizuka et al. [4] demonstrated cortical laminar necrosis and focal HMPAO SPET hyperperfusion during subacute stages of stroke-like episodes; moreover, they reported several cases of microhemorrhages and at least one case of intracortical gyral hemorrhage, suggesting that stroke-like episodes are characterized by increased capillary permeability and focal hyperemia [5]. Consistently, vasogenic edema has been reported during stroke-like episodes [6]. Anyhow, in our case, the aspect of the lesion does not suggest the hemorrhagic transformation of a stroke-like episode; this also considering that the hematoma was confined in a specific vascular territory, which is not a characteristic of stroke-like episodes [4]. Conversely, we cannot exclude a hemorrhagic coincidental event in a patient carrying the A3243G mutation with a subclinical MELAS phenotype. However, mitochondrial angiopathy in E. Saracchi L. Tremolizzo J. C. DiFrancesco L. Brighina G. Costantino B. Frigeni M. Brioschi M. L. Piatti L. Fumagalli L. Marzorati N. A. Curto C. Ferrarese Department of Neurology, S. Gerardo Hospital, Monza, Italy

Nonalcoholic Wernicke Encephalopathy with Extensive Cortical Involvement: Cortical Laminar Necrosis and Hemorrhage Demonstrated with Susceptibility-Weighted MR Phase Images: Fig 1.

Wernicke encephalopathy (WE) is an acute neuropsychiatric condition due to an initially reversible brain lesion caused by depleted intracellular thiamine levels in neurons.[1][1] Most of the affected patients are malnourished alcoholics; however, WE has also been associated with other conditions.

Cortical Laminar Necrosis in an Infant with Severe Traumatic Brain Injury

Cortical laminar necrosis appears as hyperinense lesions with a laminar pattern on T1 weighted magnetic resonance (MR) imaging, without signs of hemorrhage or calcification on T2 weighted MR imaging or computed tomography. It has been reported to be associated with hypoxia, metabolic disturbances, drugs, and infections. We present a 12 month-old male infant who suffered diffuse brain injuries following car accident and showed laminar necrosis of cortex.

Brainstem: Neglected Locus in Neurodegenerative Diseases

The most frequent neurodegenerative diseases (NDs) are Alzheimer’s disease (AD), Parkinson’s disease (PD), and frontotemporal lobar degeneration associated with protein TDP-43 (FTLD–TDP). Neuropathologically, NDs are characterized by abnormal intracellular and extra-cellular protein deposits and by disease-specific neuronal death. Practically all terminal stages of NDs are clinically associated with dementia. Therefore, major attention was directed to protein deposits and neuron loss in supratentorial (telencephalic) brain regions in the course of NDs. This was also true for PD, although the pathological hallmark of PD is degeneration of pigmented neurons of the brainstem’s substantia nigra (SN). However, PD pathophysiology was explained by dopamine depletion in the telencephalic basal ganglia due to insufficiency and degeneration of the projection neurons located in SN. In a similar line of argumentation AD- and FTLD-related clinical deficits were exclusively explained by supratentorial allo- and neo-cortical laminar neuronal necrosis. Recent comprehensive studies in AD and PD early stages found considerable and unexpected involvement of brainstem nuclei, which could have the potential to profoundly change our present concepts on origin, spread, and early clinical diagnosis of these diseases. In contrast with PD and AD, few studies addressed brainstem involvement in the course of the different types of FTLD–TDP. Some of the results, including ours, disclosed a higher and more widespread pathology than anticipated. The present review will focus mainly on the impact of brainstem changes during the course of the most frequent NDs including PD, AD, and FTLD–TDP, with special emphasis on the need for more comprehensive research on FTLDs.

Ingestion of gasoline in a suicide attempt: an uncommon cause of bilateral basal ganglia T1 hyperintensities

A 70-year-old man with a 27-year history of psychosis and hypertension was brought comatose to our Emergency Room following a suicide attempt with oral unleaded gasoline ingestion that was witnessed and described by his neighbors. His neurological examination disclosed a Glasgow Coma Scale Score of 8 (eye opening response 2 points, verbal response 2 points, motor response 2 points), with normal brainstem reflexes and spontaneous breathing. The subject was treated with a gastric lavage (gastric content, almost 2 l of gasoline) and activated charcoal. A baseline brain CT was unremarkable, while his liver function tests were normal. His neurological status did not improve and was transferred to the Intensive Care Unit of our institution where severe aspiration pneumonia was diagnosed. A brain MRI was performed at 16 days following his suicide attempt. Bilateral hyperintensities affecting the globus pallidus and the caudate nucleus were shown on axial T1, T2 and FLAIR sequences (Fig. 1). The patient expired 3 weeks later because of his aspiration pneumonia. Lesions with high signal intensity on spin-echo Tl-weighted MR images are unusual and are associated with relatively few entities, including lipids, calcification, high protein count, laminar necrosis in cerebral infarction, neoplasms and paramagnetic substances such as methemoglobin, melanin, copper and manganese [1]. Manganese, in particular, is considered a neurotoxic agent causing movement and psychological disorders characterized by rigidity, tremor and hypokinesia in workers involved in the alloy industry (miners, welders, smelters), in patients receiving long-term total parenteral nutrition and in hepatic encephalopathy patients with portal liver shunts [1–3]. Since the introduction of MRI in clinical practice, bilateral symmetric high signal intensities at the globus pallidus and substantia nigra have been well described in patients with manganese neurotoxicity. The most plausible explanation for the increased T1 signal intensity is a rise in manganese concentration that has paramagnetic properties in the central nervous system (CNS), with preferential deposition in the globus pallidus. Methylcyclopentadienyl manganese tricarbonyl (MMT) is an octane-improving additive to unleaded gasoline, which has recently attracted attention with regard to the prospect of worldwide manganese exposure as increases in environmental manganese concentrations have been recorded relative to traffic density [4]. We postulate that in the present case oral ingestion of large quantities of gasoline resulted in MMT absorption, which in turn resulted in increased CNS manganese concentrations leading to bilateral T1 basal ganglia hyperintensities. However, it should be noted that manganese concentrations were not measured in blood, while we have no neuropathological confirmation of manganese deposition in globus pallidus. In conclusion, the present case highlights bilateral T1 basal ganglia hyperintensities related to oral ingestion of gasoline in a suicide case and underscores manganese neurotoxicity as the potential underlying cause of this uncommon neuroimaging presentation. G. Tsivgoulis (&) I. Heliopoulos K. Vadikolias C. Piperidou Department of Neurology, Democritus University of Thrace, University Hospital of Alexandroupolis, Kapodistriou 3, Nea Xili, 68100 Alexandroupolis, Greece e-mail: tsivgoulisgiorg@yahoo.gr