Lithium-induced subacute diencephalic angio endotheliopathy.

A 65-years-old woman with bipolar affective disorder presented to our ED with a severe lithium intoxication and the recent onset of confusion, clumsiness, and tremors. Symptoms worsened to stupor and anarthria immediately after hospital admission. Gadolinium-enhanced brain Magnetic Resonance Imaging (MRI) showed signal hyperintensity involving both thalami in T2weighted (T2w)/Fluid Attenuated Inversion Recovery sequences (right > left), restricted areas of proton diffusivity at the level of both occipital lobes and a sharp contrast enhancement of thalami, rhombencephalon, and of leptomeninges from either the temporal, parietal, occipital lobes as well as from the cerebellar folia (right > left). These findings were consistent with a severe form of Posterior Encephalopathy known as Subacute Diencephalic Angio Endotheliopahty (SDAE). In addition, Magnetic Resonance Angiography revealed thrombosis of the right transverse and sigmoidal sinuses up to confluence with the jugular vein. The MRI picture resolved one month later after a course of high dosage dexamethasone. The patient deceased one month after discharge, mainly due to Diabetes Insipidusassociated hypernatremia. Dissecting the "Pandora's box" represented by complex MRI findings (SDAE and sinus thrombosis) in lithium-induced neurotoxicity is fundamental in timely recognizing this threating but potentially reversible clinical picture.

A very rare cause of leukoencephalopathy: Lymphomatosis cerebri.

Leukoencephalopathy is a common finding on Magnetic Resonance Imaging (MRI), particularly in the elderly. A differential diagnosis may represent a very bet for clinicians when clear elements for diagnosis are lacking. Diffuse infiltrative "non mass like" leukoencephalopathy on MRI may represent the presentation of a very rare aggressive condition known as lymphomatosis cerebri (LC). The lack of orienting data, such as contrast enhancement on MRI or specific findings on examination of Cerebrospinal Fluid (CSF) or blood tests, may even far more complicate such a difficult diagnosis and orientate toward a less aggressive but time-losing mimic. A 69-old man initially presented to the Emergency Department (ED) complaining the recent appearance of unsteady walking, limitation of down and upgaze palsy, and hypophonia. Brain MRI revealed the presence of multiple, confluent hyperintense lesions on T2/Flair Attenuated Imaging Recovery (FLAIR) sequences involving either the withe matter of the semi-oval centres, juxtacortical structures, basal ganglia, or bilateral dentate nuclei. DWI sequences showed a wide restriction signal in the same brain regions but without any sign of contrast enhancement. Initial 18F-labeled fluoro-2-deoxyglucose positron emission tomography (FDG PET) and CSF studies were not relevant. Brain MRI revealed a high choline-signal, abnormal Choline/ N-Acetyl-Aspartate (NAA), and Choline/Creatine (Cr) ratios, as well as reduced NAA levels. Finally, a brain biopsy revealed the presence of diffuse large B-cell lymphomatosis cerebri. The diagnosis of lymphomatosis cerebri remains elusive. The valorisation of brain imaging may induce clinicians to suspect such a difficult diagnosis and go through the diagnostic algorithm.

Bringing door-to-needle times within the European benchmarks results in better stroke patients outcomes in a spoke hospital from the Apulian Region.

INTRODUCTION: Door-to-needle time (DNT) is a key factor in acute stroke treatment success. We retrospectively analysed the effects of a new protocol aimed at reducing treatment delays in our single-centre observational series over a 1-year period (from October 1st 2021 to September 30th 2022). METHODS: The time frame was divided into two semesters as a new protocol was started at the beginning of the second semester to ensure a rapid evaluation, imaging, and intravenous thrombolysis in all stroke patients attending our spoke-hospital serving 200,000 inhabitants. Logistics and outcome measures were obtained for each patient and compared before and after implementation of the new protocol. RESULTS: A total of 215 patients with ischemic stroke attended our hospital within a 1-year period (109 in the first semester, 96 in the second semester). Seventeen percent and 21% of all patients underwent acute stroke thrombolysis in the first and second semesters, respectively. DNTs were strongly reduced in the second semester (from 90 to 55 min), bringing this value below the Italian and European benchmarks. This resulted in better short-term outcomes (an average of 20%) as measured by both Δ NIHSS scores at 24 h and at discharge with respect to baseline.

Motor Phenomena Associated With Leucine-Rich Glioma-Inactivated (LGI1) Emi-Encephalitis.

LGI1 encephalitis is a rare immune-mediated brain disorder. Its typical features include faciobrachial dystonic seizures (FBDS), startle reactions, chorea, myoclonus, atypical parkinsonism, cogni-tive impairment, and personality changes. We report the case of a 57-year-old woman presenting with distinct patterns of involuntary movements, including faciobrachial dystonic spasms, dyskinetic movements, and chorea. Magnetic resonance imaging (MRI) and tests on blood and cerebrospinal fluid (CSF) demonstrated encephalitis involving the right temporal lobe and caudate nucleus and associated with LGI1-antibody. LGI1 encephalitis may present with simultaneous distinct patterns of movement disorders depending on the cortical and subcortical structures involved in the disease.

Lemierre's syndrome complicated by cerebral venous sinus thrombosis: A life threatening and rare disease successfully treated with empiric antimicrobial therapy and conservative approach.

Lemierre's syndrome (LS) is a "forgotten" condition characterized by septic thrombophlebitis of the jugular vein that follows an otolaryngological infection. Fusobacterium necrophorum is the aetiological agent responsible for the syndrome in adolescents and young adults whereas in older people even common bacteria are involved. Complications arise from spreading of septic emboli distally, i.e. to the brain, lungs, bones and internal organs everywhere in the body. We report a middle-aged woman who presented with headache and bilateral sixth cranial nerve palsy following a sphenoidal sinusitis and left mastoiditis. Imaging revealed thrombotic involvement of the left internal jugular vein as well as of several cerebral venous sinuses thrombosis (CVT). Currently, precise management protocols of LS with CVT complication do not exist although a combination of macrolides and second or third-generation cephalosporins, as well as anti-coagulants represent the mainstream of therapeutics. Surgical drainage is associated to remove septic foci but is burdened by severe complications and side effects. Complete recovery was achieved following pharmacological treatment in our patient. This report adds further evidence that LS complicated by CVT may be effectively treated adopting a conservative approach thus avoiding surgical drainage and severe complications.

Posterior reversible encephalopathy syndrome due to arterial hypertension may mark the onset of the symptomatic phase in Huntington's disease.

Autonomic dysregulation of cardiovascular functions marks early Huntingtons disease (HD). Blood-brain barrier (BBB) is dysfunctional in HD. A 37-year-old female carrying 41 CAG triplets in the huntingtin gene acutely presented with a multifaceted syndrome attributable to posterior reversible encephalopathy syndrome (PRES). Syndrome was associated with arterial hypertension (AHT). The syndrome fully recovered both by imaging and clinical signs after normalization of arterial pressure during hospitalization. Immediately after hospital discharge, the patient developed a complex psychiatric syndrome and choreic movements that represented conversion to the symptomatic phase of HD. A one-year later follow up clearly showed the patient had developed the symptomatic stage of HD by presenting both psychiatric symptoms and choreic movements. Onset of AHT may represent an early premonitory signal of HD becoming manifested. Induction of PRES might be associated with BBB impairment in HD.

Functional Rabbit Syndrome: A Case Report.

Rabbit Syndrome is a rare involuntary movement occurring in 1.5-4.4% of patients receiving antipsychotics and characterized by rapid, regular movements (4-6 Hz) of the oral and masticatory musculature resembling the chewing motions of a rabbit. Herein we describe a middle-aged woman who presented with a rabbit syndrome characterized by several clues of psychogenicity such as sudden onset, distractibility, variability and complete "miracolous" remission.

The progression of posterior cortical atrophy to corticobasal syndrome: lumping or splitting neurodegenerative diseases?

BACKGROUND: Posterior cortical atrophy is a clinical syndrome that is characterized by the progressive loss of visuospatial integration and is associated with neurodegenerative conditions. CASE REPORT: We describe a 60-year-old female with simultanagnosia, oculomotor apraxia, and optic ataxia for which she received an initial clinical diagnosis of posterior cortical atrophy. Three years later, she developed Balint's syndrome, Gerstmann's syndrome, left alien hand syndrome, smooth asymmetric (left) rigidity, cortical sensory loss, and spontaneous myoclonic jerks of the left arm, which suggested a final diagnosis of corticobasal syndrome. DISCUSSION: This case report indicates that corticobasal syndrome may present with visuospatial deficits.

Change in Non-motor Symptoms in Parkinson's Disease and Essential Tremor Patients: A One-year Follow-up Study.

BACKGROUND: Non-motor symptoms (NMS) in Parkinson's disease (PD) differ from those in essential tremor (ET), even before a definitive diagnosis is made. It is not clear whether patient's knowledge of the diagnosis and treatment influence their subsequent reporting of NMS. METHODS: 1 year after a clinical and instrumental diagnosis, we compared the motor impairment (Movement Disorders Society (MDS)-Unified Parkinson's Disease Rating Scale-III) and non-motor symptoms (NMSQuest) in PD (n = 31) and ET (n = 21) patients. RESULTS: PD patients reported more NMS than did the ET patients (p = 0.002). When compared to their baseline report, at follow-up, PD patients reported less nocturia (p = 0.02), sadness (p = 0.01), insomnia (p = 0.02), and restless legs (p = 0.04) and more nausea (p = 0.024), unexplained pain (p = 0.03), weight change (p = 0.009), and daytime sleepiness (p = 0.03). When compared to their baseline report, ET patients reported less loss of interest (p = 0.03), anxiety (p = 0.006), and insomnia (p = 0.02). Differences in reported weight change (p<0.0001) and anxiety (p = 0.001) between PD and ET patients were related to pharmacological side effects or to a reduction in the ET individuals. DISCUSSION: The reporting of NMS is influenced by subjective factors, and might vary with the patient's knowledge of the diagnosis or the effectiveness of treatment.

Do non-motor symptoms in Parkinson's disease differ from essential tremor before initial diagnosis? A clinical and scintigraphic study.

BACKGROUND: Non-motor symptoms (NMS) in Parkinson's disease (PD) are common, increase the patients' disability and have a significantly negative impact on their quality of life. Essential tremor (ET) is also affected by non-motor symptoms and often enters into the differential diagnosis with PD. Brain scintigraphy with [(123)I]ß-CIT SPECT is a technique used to facilitate differential diagnosis between PD and ET. METHODS: We evaluated both motor impairment (MDS-UPDRS-III) and non-motor symptoms (NMSQuest) in patients who underwent a [(123)I]ß-CIT SPECT examination for diagnostic purposes. Both the clinical and the scintigraphic data obtained from the selected PD (n = 31) and ET (n = 22) patients were compared. RESULTS: We did not detect a significant difference in the total number of NMS reported by either PD (10.4 ± 4.9) or ET patients (8.41 ± 3.3). PD patients reported more drooling (29%), hyposmia (32.2%), hallucinations (19.3%), difficulty in concentrating (51.6%), orthostatic dizziness (67.7%), falling (19.3%), vivid dreams (32.2%), REM sleep behavior disorder (58%), and diplopia (22.5%) compared with ET patients. PD patients who complained of drooling, orthostatic dizziness, and diplopia had greater denervation of the caudata than did the PD patients who did not report the same symptoms. The differences observed were not associated with differences in age, sex, UPDRS-III score, and the presence/absence of tremor. CONCLUSIONS: The declaration of non-motor symptoms is influenced by subjective factors that are widely suggestible. When analyzed early and before receiving a definitive diagnosis, PD patients complain of specific symptoms that seem to depend on different pathogenetic mechanisms.

Hairdresser's Dystonia: An Unusual Occupational Dystonia.

BACKGROUND: Adult-onset focal dystonias (AOFDs) are non-task-specific or task-specific and may spread to other body segments of affected patients. CASE REPORT: We report the case of a barber with non-task-specific craniocervical dystonia and a new occupational focal hand dystonia (while using scissors). DISCUSSION: Different AOFDs may develop and coexist in the same "vulnerable" patient. Hairdresser's dystonia is a rare task-specific dystonia.

Dopaminergic modulation of CD4+CD25(high) regulatory T lymphocytes in multiple sclerosis patients during interferon-ß therapy.

OBJECTIVE: We investigated dopaminergic inhibition of CD4+CD25(high) regulatory T lymphocytes (Treg) in relapsing-remitting multiple sclerosis (MS) patients treated with interferon (IFN)-ß. METHODS: MS patients were prospectively studied at baseline and during 1 year of IFN-ß, and compared with healthy controls (HCs). Treg were separated by immunomagnetic sorting and the effect of dopamine (DA) on Treg was assessed in coculture experiments with homologous effector T lymphocytes (Teff). Tyrosine hydroxylase (TH), dopaminergic receptors (DR) D3 and D5, and forkhead box protein P3 (FoxP3) mRNA were assessed by real-time PCR. Circulating CD4+ T cell subsets were assessed by flow cytometry. RESULTS: In coculture experiments, Treg inhibition of Teff proliferation was reduced by DA in HCs and completely abolished in MS patients at baseline. However, in patients after 12 months of IFN-ß, Teff proliferation was impaired and DA had no more effect on Treg. In comparison to cells from HCs, Treg from MS patients at baseline had increased mRNA for DR D5 and TH (but not for DR D3). During treatment with IFN-ß, both DR D5 and TH mRNA decreased down to values lower than those of cells from HCs. In comparison to HCs, MS patients had a higher frequency of circulating Treg, both at baseline and after IFN-ß, while FoxP3 mRNA levels in Treg were similar in patients and HCs and did not show major changes during IFN-ß. CONCLUSIONS: Dopaminergic inhibition of Treg in MS patients is suppressed during IFN-ß treatment. Treg play a key role in the suppression of autoimmunity, thus the effect may have a therapeutic repercussion.