Mariam Annan

Sinking skin flap syndrome (or Syndrome of the trephined): A review

Abstract Background. Bone defects of the skull are observed in various pathological conditions, including head trauma and conditions requiring surgery of the skull. Independent of the consequences of the original aetiology that necessitated the craniectomy, the bone defect alone may be the cause of the symptoms, called ‘trephined syndrome’ or ‘sinking skin flap syndrome’. Despite the early recognition of neurological symptoms directly linked to craniectomy, the description of this syndrome has often relied on a small series or single clinical case reports. Objectives. To list the previously reported symptoms of SSFS. Data sources. We selected the references for this review by searching PubMed, focusing on articles published prior to June 2013 and using references from relevant articles. Study eligibility criteria. We used the following search terms: ‘trephined syndrome’, ‘syndrome of the trephined’, ‘Sinking skin flap’, and ‘sinking skin flap syndrome’. There were no language restrictions. The final reference list was generated on the basis of its relevance to the topics covered in this review. Conclusions. Clinicians need to be aware of sinking skin flap syndrome and to look for abnormal neurological developments in patients with craniectomy in order to avoid unnecessary testing and to prevent its occurrence. Accordingly, cranioplasty can be undertaken as soon as necessary.

Susceptibility–Diffusion Mismatch in Hyperacute Stroke: Correlation with Perfusion–Diffusion Mismatch and Clinical Outcome

BACKGROUND A prominent vein (PV) on susceptibility-weighted imaging (SWI) was recently proposed to be a marker of the penumbra. We aimed to compare the utility of SWI and perfusion-weighted imaging (PWI) sequences for the evaluation of the penumbra in hyperacute middle cerebral artery (MCA) stroke, and to determine whether SWI-DWI mismatch is a neuroimaging marker of clinical outcome. METHODS A total of 149 consecutive patients with MCA stroke were prospectively enrolled. Magnetic resonance imaging (MRI) was performed within 6 hours of the onset of stroke. The ASPECTS values on diffusion-weighted imaging (DWI), PWI (delayed mean transit time), and SWI (visualization of PVs) were calculated by 2 independent raters. Correlation between PWI-ASPECTS and SWI-ASPECTS was calculated with the Pearson coefficient. Reliability of the PV rating system was calculated by an intraclass correlation coefficient (ICC). Favorable outcome was defined as a modified Rankin Scale score of 0-2 at 3 months for the 88 patients who received thrombolytic therapy. RESULTS The ASPECTS-SWI and ASPECTS-PWI scores showed a good correlation (Pearson coefficient of .69, P <.001). The reproducibility between the findings of the junior and the senior radiologists was excellent with an ICC of .89 (confidence interval of 95% (IC95): .85-.92, P <.001). However, neither SWI-DWI mismatch nor PWI-SWI mismatch was associated with clinical outcome. CONCLUSION SWI and PWI were complementary but not commutable for the assessment of the penumbra. Susceptibility-diffusion mismatch was not found in this study to have predictive value for stroke outcome.

Idiopathic Parkinson’s disease phenotype related to C9ORF72 repeat expansions: contribution of the neuropsychological assessment

BackgroundExpanded GGGGCC hexanucleotide repeats in the non-coding region of the C9ORF72 gene was recently identified as being responsible for over 40% of the cases of amyotrophic lateral sclerosis associated with frontotemporal lobar degeneration, in various extrapyramidal syndromes including supranuclear gaze palsy and corticobasal degeneration, and in addition, has been found to be a rare genetic cause of isolated Parkinsonism. To our knowledge, there is no published data concerning the neuropsychological evaluation of patients diagnosed with idiopathic Parkinson’s disease related with C9ORF72 repeat expansions.Case presentationWe report the results of the comprehensive neuropsychological evaluation in a newly described case in the literature (the sixth) of a patient presenting isolated idiopathic Parkinson’s disease associated with C9ORF72 repeat expansions.The decrease in the patient’s prefrontal functions resulted in a slight decrease in global efficiency. These abnormalities did not appear to be different, with respect to the deficit observed and the intensity of the cognitive impairment, from those classically observed in cases of sporadic idiopathic Parkinson’s disease. Our patient also exhibited a significant impairment in visual gnosis.ConclusionsIf confirmed in other patients, visuoperceptive deficits in idiopathic Parkinson’s disease could represent a red flag that should prompt the clinician to perform addition diagnostic procedures. A thorough neuropsychological assessment may prove to be useful for detecting idiopathic Parkinson’s disease in patients who are suspected of having repeat abnormalities of C9ORF72 expansions.

Appearance of post ictal psychosis after radiosurgical damage in the temporal lobe

Mariam Annan *, Fabrice Bartolomei , Julien Biberon , Jean-Philippe Cottier , Bertrand De Toffol a,b,f a CHU Bretonneau, Service de neurologie, CHRU Bretonneau, 2 Boulevard Tonnelle, 37000 Tours, France b Faculte de medecine Francois Rabelais, Tours, France CHU Timone, Service de Neurophysiologie Clinique, Assistance Publique des Hopitaux de Marseille, Marseille F-13005, France d INSERM U1106, Marseille, France e CHU Bretonneau, Service de radiologie, 2 Boulevard Tonnelle, 37000 Tours, France f INSERM U 930, Tours, France

Functional Outcome of Hemorrhagic Transformation after Thrombolysis for Ischemic Stroke: A Prospective Study

Background/Aims: Hemorrhagic transformation (HT) is usually taken into account when symptomatic, but the role of asymptomatic HT is not well known. The aim of our study was to evaluate the link between HT after thrombolysis for ischemic stroke and functional outcome at 3 months, with particular emphasis on asymptomatic HT. Methods: Our study was performed prospectively between June 2012 and June 2013 in the Stroke Unit of the University Hospital Center of Tours (France). All patients treated with intravenous thrombolysis were consecutively included. HT was classified on susceptibility-weighted imaging (SWI) with 3-tesla MRI at 7 ± 3 days after treatment. We evaluated functional outcome at 3 months using the modified Rankin Scale (mRS). Dependency was defined as an mRS score of ≥3. Results: After 1 year, 128 patients had received thrombolytic therapy for ischemic stroke, of whom 90 patients underwent both 3-tesla MRI and SWI at day 7. Fifty-two had HT, including 8 symptomatic cases. At 3 months, 68% of those patients were dependent compared to 31% of patients without HT [OR 4.6 (1.9-11.4), p = 0.001]. In asymptomatic HT, the rate was 62% [OR 3.5 (1.4-8.9), p = 0.007], but did not reach significance after adjustment for stroke severity. Discussion: Our study found no statistically significant effect of HT on outcome after adjustment for initial stroke severity. However, the innocuousness of HT is not certain, and only few studies have already highlighted the increased risk of dependency. Using 3-tesla MRI with SWI allows us to increase the detection rate of small hemorrhage. Conclusion: HT after thrombolysis is very frequent on SWI, but the initial stroke severity is an important predictor to assess the role of HT for patient outcome.

Deterioration following craniectomy

A 35-year-old right-handed man was admitted to the rehabilitation unit, 4 weeks after a traumatic brain injury. He had been found unconscious in the street several hours after drinking with friends. On first admission to the emergency unit, his Glasgow Coma Scale score was 6 out of 15. His CT scan of head showed an occipital condyle fracture, an acute right subdural haematoma, bifrontal intraparenchymal and subarachnoid haemorrhage, and right hemisphere oedema. A few hours following intensive care unit admission, he developed a right reactive mydriasis. His intracranial pressure remained high despite osmotherapy, and he underwent decompressive craniectomy with subdural …

Case report of chronic cough and legs edema of an 84-year-old man: When the solution is found in the nails

Older adults often present independent and interlinked multiple chronic conditions. Occasionally very different and frequent symptoms might be related with rare syndromes. An 84-year-old man presented at University Hospital Center of Tours, Tours, France, with lymphoedema in the legs and a chronic cough. His past medical history included a mild hypertension, lower limb peripheral arterial disease, sciatic nerve palsy, progressive primary aphasia and gout. His medications included irbesartan (150 mg/day), venlafaxine (37.5 mg/day), febuxostat (80 mg/day), rosuvastatine (10 mg/day), esomeprazole (40 mg/day) and aspirin (160 mg/day). The patient had for 2 years the concomitant appearance of a chronic cough and lymphoedema in the lower limbs. The cough was described as a loose cough without expectorations, mostly nocturnal, with coughing fits causing insomnia. Several episodes of acute sinusitis were also noticed. There was no abnormal sound at pulmonary auscultation or cardiac abnormality. Blood cell counts were normal, and B-type natriuretic peptide and an ionogram were both in the normal range. A chest X-ray and lung function studies were also normal. Thoracic X-ray tomodensitometry showed nodular calcifications without abnormality of the parenchyma. Cutaneous examination showed lower limbs, non-tender, pitting lymphoedema (Fig. 1a). All of the 20 nails appeared to be markedly thickened and presented a yellow discoloration (Fig. 1b,c). The patient needed to cut his nails every three months. The appearance of the nail discoloration suggested a few etiologies. Associations with several medical conditions (hemodialysis, malignancy, connective tissue disease, tuberculosis, snake bite, xanthogranulomatous pyelonephritis, hypothyroidism and diabetes) or drugs (bucillamine, 5-fluorouracil, gold therapy, phenazopyridine) have been described. Paraclinical investigations include thyroid-stimulating hormone as well as creatinin and glucose levels; thoracic as well as abdominal imaging dependent on the clinical context are carried out to document neoplasm. Most often, these etiologies are not found, and therefore, the diagnosis that might be given is referred to as “yellow nails syndrome.” This rare entity was first described in the 1960s as the association of yellow nails and/or lymphoedema and/or chronic respiratory manifestations. The