Irreversible hypersomnolence after bilateral thalamic infarction

info:eu-repo/semantics/publishedVersio

Mirrored-self misidentification in a patient with probable Alzheimer dementia

A 77-year-old-man who was a retired primary school teacher with probable Alzheimer dementia diagnosed after 2 years of progres sive short-term memory loss and predominant hippocampal atro phy onmagnetic resonance scan of the brain was brought to the out patient neurology clinic by his wife because of persistent ab normal behavior. The caretaker reported that he was frequently puzzled and astonished by his own mirror image. His medical his tory was remarkable for controlled hypertension and type 2 diabe tes. He did not have any personal or family history of psychiatric dis ease. There were no recent changes with the patient’s regular medications (rivastigmine, 9.5 mg daily transdermally; enalapril; and metformin). His wife described him as being a very quiet and relaxed person until the start of restrictions imposed by the COVID-19 pandemic, when he started with complaints of seeing a strangeman whenever he saw his face reflection in the mirror.info:eu-repo/semantics/publishedVersio

Should we mind for late neurologic manifestations from novel coronavirus?

We read with great interest the article published by Montalvan et al. [1] reviewing the neurological manifestations of COVID-19 and other coronavirus infections. The review was focused on the acute complications described in humans and in animal models of coronavirus infection.info:eu-repo/semantics/publishedVersio

Pilocytic astrocytoma

Pilocytic astrocytoma (PA) is a benign tumour of childhood, often located in deep midline structures such as the brainstem and the cerebellum.1 Gross surgical resection is curative in the majority of patients.2 We report a case of recurrent PA diagnosed after an acute confusional state and rightsided spastic hemiparesis in a previously healthy 38-year-old woman. Her initial brain CT showed a left temporal mass causing obstructive hydrocephalus (figure 1A–C). Acute surgical extraction was performed (figure 1D) and the biopsy confirmed the diagnosis of PA grade I (WHO). The patient remained clinically asymptomatic for 1 year, when a progressive headache emerged. At this time, the neurological examination disclosed the presence of dysarthria and right-sided hemiparesis with facial involvement. Brain MRI showed the presence of recurrent PA (figure 1E, F). The patient again underwent surgery due to the mass effect and brainstem compression caused by the tumour, and the diagnosis of PA was reconfirmed histologically. The patient fully recovered after the intervention. PA occurs commonly in the first 2 decades of life.1 Information on the characteristics of PA in adulthood is scarce due to its rarity. Some studies indicate the adult prognosis to be similar to that in children, while others indicate that PA may show aggressive behaviour with tumour recurrence and death.2 Anaplastic features are associated with worse prognosis, but little is known about the value of genetic characterisation in adulthood PA.3 In conclusion, this case offers a unique description of adult onset PA in an atypical location outside the midline structures, and also provides an example of early recurrence

On the plausibility of late neuropsychiatric manifestations associated with the COVID-19 pandemic

Recognition of the association between acute and subacute different neuropsychiatric manifestations with the infection by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the cause of the ongoing coronavirus disease 2019 (COVID-19) pandemic, is progressively being recognized.info:eu-repo/semantics/publishedVersio

Dropped head and man-in-barrel syndrome in amyotrophic lateral sclerosis

We report a case of progressive symmetric brachial weakness followed by cervical muscle weakness. The electromyogram confirmed the diagnosis of amyotrophic lateral sclerosis. After 3 years the patient remained able to walk unassisted and without significant bulbar manifestations or upper neuron signs. The concomitant presence of dropped head syndrome and man-in-barrel syndrome in an amyotrophic lateral sclerosis patient makes our case unique.info:eu-repo/semantics/publishedVersio

Caucasian Familial Moyamoya Syndrome With Rare Multisystemic Malformations

Moyamoya disease is an idiopathic progressive steno-occlusive disorder of the intracranial arteries located at the base of the brain. It is associated with the development of compensatory extensive network of fine collaterals. Moyamoya disease is considered syndromic when certain genetic or acquired disorders such as polycystic kidney disease, neurofibromatosis, or meningitis are also present. Although the genetic contribution in moyamoya is indisputable, its cause and pathogenesis remain under discussion. Herein, we report a rare occurrence of moyamoya syndrome in two European Caucasian siblings in association with unusual multisystemic malformations (polycystic kidney disease in one, and intestinal duplication cyst in the other). The karyotype was normal. No mutation in the RFN213 gene was found, and none of the HLA types linked to moyamoya disease or described in similar familial cases were identified. By describing these multisystemic associations, polycystic kidney disease for the second time, and intestinal malformation for the first time in the literature, our report expands the phenotypic variability of moyamoya syndrome. The coexistence of disparate malformations among close relatives suggests an underlying common genetic background predisposing to structural or physiological abnormalities in different tissues and organs

IgG4: related disease presenting with headache and papilloedema

A previously healthy man aged 57years presented with a 4-week duration of predominant decubitus daily holocranial headache after minor head injury. Apart from unintentional weight loss of 8 pounds, there were no associated constitutional or systemic manifestations.The generalandneurologicalexamination was unremarkable. The funduscopy showed bilateral grade 2 papilloedema. Brain magnetic resonance (MRI) findings are shown in figure 1A–E. The brain MRI showed the presence of diffuse dural thickening (figure 1B–E) hypointense on unenhanced T1 imaging (figure 1B–F), with dural enhancement on T1-weighed imaging (figure 1B–F). The opening cerebrospinal fluid (CSF) pressure was 380mm Hg. With the exception of serum-elevated IgG4 immunoglobulin (IgG4=302 mg/dL) and elevated CSF protein level (68 mg/dL), the wide and extensive complementary study including body positron emission tomography scan, laboratory blood/CSF work-up was negative, excluding neoplastic, infectious, autoimmune and collagen vascular disorders. Meningeal biopsy showed fibrous thickening with moderate and predominant B and T cells inflammatory infiltrate. The patient improved with spinal taps evacuation and methylprednisolone treatment. Intracranial hypertrophic pachymeningitis is a severe and rare manifestation in the spectrum of IgG4-related diseases.12Biopsy of meninges is the gold standard for the diagnosis.3 Exclusion of systemic inflammatory disorders and demonstration elevated serum IgG4 can support the diagnosis in patients with typical diffuse dural thickening.13The disease respondsto immunosuppressive drugs such as corticosteroids, methotrexate, azathioprine, mycophenolate or cyclophosphamide.12Mass effect due to dural thickening can cause progressive neurological debilitation,blindness and cerebellarataxia.In the case of symptomatic hydrocephalus, surgical exploration for decompression maybe require

Ischemic stroke and subarachnoid hemorrhage following Epstein-Barr virus infection

The epidemiological link between inflammation, infection, and acute ischemic stroke (AIS) is well known.1 Transient prothrombotic status and vasculitis are possible explanatory factors.1 Aneurysm formation in association with central nervous system infections is occasionally reported.2 Ischemic or hemorrhagic complication in relation to neurotropic virus infections, including Epstein-Barr Virus (EBV) are rarely reported in adults. Thus, we report here an extremely rare case of ischemic and hemorrhagic cerebrovascular complications in the context of EBV neuroinfection.info:eu-repo/semantics/publishedVersio

Lung caught in Nilutamide treatment

An 86-year-old male patient was attended with dyspnoea for 2 weeks, with progressive worsening, dry cough and decreased appetite. He had a personal history of prostate cancer diagnosed 2 years before and dyslipidaemia. He denied smoking history, exposure to inhaled toxic substances or family history of chronic lung disease. The patient was receiving treatment with rosuvastatin 10 mg and nilutamide 150 mg which was started 1 month earlier. On physical examination we found the patient with globally decreased breath sounds and crackles at the base of the right hemithorax. Laboratory tests showed no other abnormalities besides C-reactive protein 69 g/L and slight hypoxaemia. The chest X-ray showed bilateral interstitium infiltrates with slight effacement of the costophrenic angles and elevation of the right hemicupula (figure 1A). The thorax CT scan was consistent with interstitial pneumonitis (figure 1B, C). Serological tests for viral atypical organisms, antinuclear antibodies, ACE inhibitors and antineutrophil cytoplasmic antibodies were carried out but with negative results. Pulmonary function tests, alveolar lavage and lung biopsy were not performed. The treatment with nilutamide was discontinued and the patient was started on bronchodilator therapy and prednisolone 40 mg/day with clinical improvement after 3 days of hospitalisation and discharge from hospital at day 14 with normal clinical observation, gasimetric normalisation, negative RCP and improved chest X-ray imaging. Nilutamide is a non-steroidal antiandrogen, which competitively inhibits the binding of androgens to the androgen receptor.1 Interstitial pneumonitis is an extremely rare, although reversible, adverse reaction to non-steroidal antiandrogen treatment, being described in 1–2% of the patients taking the drug.2 The mechanisms by which non-steroidal antiandrogens cause pulmonary disease remain unclear.3 The onset of symptoms was reported 2–8 months after the beginning of the treatment.2 The prognosis of drug-induced pneumonitis is relatively good and can be treated with the withdrawal of the drug with or without the association of steroids