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A 33-year-old man with a history of traumatic brain injury (TBI) from a few years prior, secondary to a high-speed motor vehicle accident, presented with worsening right-sided motor function. Brain MRI showed diffuse axonal injury, punctuate microbleedings, asymmetric Wallerian degeneration along the left corticospinal tract in the brainstem and haemorrhagic left superior cerebellar peduncle, all consistent with his prior TBI. Moreover, the right inferior olivary nucleus was enlarged, which is exemplified in unilateral right hypertrophic olivary degeneration (HOD), likely secondary to the haemorrhagic lesion within …

A 33-year-old homeless woman presented to the emergency room in September, 2012, with 2 days of dry cough, dyspnoea, and fever, and 1 week of pruritic rash. On examination she had a temperature of 41·0°C, blood pressure of 98/65 mm Hg, heart rate of 85 bpm, respiratory rate of 16 bpm, and O2 saturation of 47% on room air. She had a diff use papular rash with vesicles of diff erent ages with haemorrhagic crust aff ecting the trunk, extremities, face, and scalp, but sparing the palms and soles (fi gure). She had normal mental status, cranial nerves, motor, and sensory examination. Chest radiograph showed diff use bilateral airspace opacities. Within a few hours of presentation she suddenly became confused, with fl accid paralysis necessitating intubation and admission to the medical intensive care unit. Electroencephalogram showed non-convulsive status epilepticus. MRI brain showed multiple foci of punctate microhaemorrhages throughout the corpus callosum, both temporal lobes, and left posterior cingulate gyrus, consistent with infectious haemorrhagic encephalitis (fi gure). HIV testing was positive, with CD4 count 4 cells/μL (normal 348–1456 cells/μL), and viral load was 66 000 copies/mL (normal < 50 copies/mL). A skin biopsy sample stained positive for varicella zoster virus (VZV), but negative for herpes simplex virus (HSV). Zoster-associated encephalitis from disseminated VZV can develop within days of the disseminated rash, and should be strongly suspected in immunosuppressed patients. Characteristic signs of zoster-associated encephalitis include acute delirium, typical signs of stroke such as headache with acute hemiplegia, aphasia, ataxia, hemisensory loss, or visual changes, dependent on the predominant location of the VZV vasculitis. Intravenous aciclovir 10 mg/kg every 8 h for 7–10 days should be started early if there is clinical suspicion of encephalitis. Our patient was given intravenous aciclovir and multiple antiepileptic drugs to control her seizures. We treated her for VZV pneumonia, aspergillus pneumonia, propofolinduced pancreatitis, Escherichia coli urinary tract infection, and critical illness myopathy. After stabilisation in hospital she was started on antiretrovirals and pneumocystis pneumonia prophylaxis. After 2 months of rehabilitation she could walk independently and communicate without diffi culty. At last follow-up in March, 2014, she had made a near full recovery in terms of her cognitive and motor function. Lancet 2014; 384: 1698

Pineal gland cysts are typically asymptomatic, benign cysts most commonly found incidentally in adults. In rare cases, a large pineal gland cyst can be complicated by intracystic hemorrhage, which could then manifest with neurological symptoms. We report a new complication of intracystic hemorrhage in a large pineal gland cyst in a 40-year-old man with new onset seizures.

Background Bottle gourd ( Lagenaria siceraria ) is an edible plant in the Cucurbitaceae family. When extremely bitter, ingestion of bottle gourd can cause rapid onset diarrhea, vomiting, gastrointestinal bleeding, and hypotension due to release of a substance named cucurbitacin. Objective Our aim was to increase physician awareness of cucurbitacin poisoning in order to facilitate accurate diagnosis and appropriate management. Case Report Five adult patients presented with nausea, vomiting, and diarrhea within 5 to 25 min of ingesting cooked bitter bottle gourd. One patient developed severe diarrhea, hematemesis, and hypotension requiring hospitalization. All patients improved within a few days with intravenous fluids and proton pump inhibitors. To our knowledge, this is the first reported group of patients with toxicity due to ingestion of bottle gourd in the United States (US). Conclusions Physicians should be suspicious of cucurbitacin toxicity in patients who present with symptoms within minutes of ingestion of a plant in the Cucurbitaceae family. Patients should be asked if the plant tasted unusually bitter. The most common symptoms include diarrhea and hematemesis. More than half of patients develop hypotension. There is no known antidote for bottle gourd poisoning; treatment is supportive. Proton pump inhibitors should be given to patients with gastrointestinal mucosal injury.

Peutz-Jeghers syndrome (PJS) is an autosomal dominantly inherited disease. STK11/LKB1 gene germline mutations have been identified as responsible for PJS. In our study, we investigated the molecular basis of PJS and evaluated correlation between the STK11 mutations and the Chinese population.We collected three pedigrees of PJS and screened the 9 exons and their flanking intronic sequences of STK11/LKB1 gene in the probands and normal individuals in the families using polymerase chain reaction (PCR) and direct sequencing.Sequencing of the STK11 gene in the probands of 3 families revealed two novel mutations (c180C--G and c998-1002delGCAGC) in exon 1 and exon 8, respectively. The mutation of c180C--G resulted in a premature termination codon. The other mutation, a deletion of five nucleotides (998-1002delGCAGC) in exon 8, predicted to generate a translational frameshift and a termination at codon 1070.The growing number of mutations in PJS pedigrees suggests the molecular basis of PJS. STK11 gene mutation can be detected in most patients with PJS.

Dear Sirs, A 52-year-old Hispanic man was admitted to hospital with a history of seizures, right sided hemiparesis, and new onset of aphasia. He complained of seizures that began 3 years prior which were characterized by 5 min episodes of right leg twitching, spreading to his right arm. The patient had no known risk factors for seizures, and the addition of topiramate had reduced his seizure frequency to about once every 2–3 days. A previous MRI of his brain showed nonspecific fluid attenuation inversion recovery (FLAIR) hyperintensities in the left cerebral hemisphere; however, his EEG was normal. In the past, the patient had a brain biopsy for a workup of the FLAIR hyperintensities in the left hemisphere, which was inconclusive due to inadequate tissue sampling. His neurological examination was remarkable for a global aphasia, 4/5 strength on the right upper and lower extremities, and a right sided spastic gait with right sided internal circumduction of the foot. MRI scan of the brain showed extensive fluid attenuation inversion recovery (FLAIR) hyperintensities restricted to the left cerebral hemisphere, with multiple foci of nodular and ring enhancement, along with involvement of the left calvarium (Fig. 1a–c). Serum rheumatologic and infectious studies were negative outside of a positive serum Bartonella antibody titer. Cerebrospinal fluid results were unremarkable. Since the patient had a history of working with farm animals, he was treated empirically with doxycycline for 4 weeks, but with no noticeable improvement. MRI scans of the brain over the next few months showed progression of the FLAIR hyperintensities in the left hemisphere. Since the patient was a recent immigrant from Mexico, he was also treated with RIPE (rifampin, isoniazid, pyrazinamide and ethambutol) therapy for presumed tuberculosis with no noticeable improvement. Fig. 1 MRI brain revealing extensive fluid attenuated inversion recovery (FLAIR) lesions restricted to the left cerebral hemisphere (a), with multiple foci of nodular and ring enhancement (b), along with involvement of the left calvarium (c). After treatment ... After a month, the patient presented to another hospital because of worsening aphasia and headaches. An MRI scan showed further progression of the left hemispheric lesions. Four-vessel cerebral angiogram was notable for a small left middle cerebral artery saccular aneurysm and a right pericallosal anterior cerebral artery aneurysm, which were both surgically clipped, but no beading or stensoses were visualized. He then had a left frontal stereotactic brain biopsy performed revealing dense perivascular chronic inflammation with mixed T and B lymphocytes, along with reactive astrocytosis in surrounding brain. The inflammatory infiltrates were sharply demarcated from the surrounding brain and did not extend into the surrounding neuroglial tissue. The findings were consistent with primary angiitis of the CNS (PACNS) (Fig. 2a–f). The patient returned to our care and was treated with corticosteroids and cyclophosphamide. Within days after initial treatment the patient noticed improvement of speech and strength. Fig. 2 H&E stained section of a vessel, cut in longitudinal section, shows polymorphous transmural inflammatory infiltrate between the arrows (a). Note effacement of normal vessel wall by the inflammatory infiltrate (b). Sections of the ... At a follow up visit 3 months later, the patient reported near complete resolution of his aphasia, seizures and right sided hemiparesis. He has not had a seizure in over 6 months, and a follow up MRI brain revealed significant improvement of the FLAIR and significantly decreased areas of enhancement in the left hemisphere (Fig. 1d, e). Our case is the first in the literature that reports a slowly progressive, single hemispheric involvement of PACNS. The patient’s focal, unilateral lesion on MRI and slow indolent course initially raised the question of infection, tumor, or even a progressive demyelinating disease. In addition, the unusual manifestation of focal involvement of the overlying calvarium raised the possibility of granulomatous disease, such as vasculitis occurring in the setting of tuberculous meningitis. However, the diagnostic studies for tuberculosis including tuberculin skin test, sputum cultures, and cerebrospinal fluid cultures were all negative, and the MRI did not reveal the basilar meningeal thickening that might suggest tuberculous meningitis. Our patient required two brain biopsies before there was adequate sampling of the tissue and vessels involved to make the diagnosis, which is often the case in PACNS [1–3]. He also had a four vessel cerebral angiogram which revealed anterior cerebral and middle cerebral artery aneurysms, which have been described in the literature in association with PACNS [4]. This represents the first case report of focal PACNS. Glucocorticoid and immunosuppressive therapy are first-line treatments for PACNS based on extrapolations from treatment of other systemic vasculitides and from expert consensus groups, although intravenous immunoglobulin, plasmapharesis, and mycophenolate mofetil have also shown some success [5–7]. Our case describes an unusual presentation of PACNS which had an excellent response to corticosteroid and immunosuppressive therapy.