Brainstem evoked potentials and magnetic resonance imaging abnormalities in differential diagnosis of intracranial hypotension

Objective: To compare brainstem acoustic evoked potentials (BAEP)and magnetic resonance imaging (MRI)in the differential diagnosis of intracranial hypotension (IH), Chiari malformation (CM)and sensorineural hearing loss (SNHL). Methods: BAEP were recorded in 18 IH, 18 CM, 20 SNHL patients and 52 controls. MRI were acquired in all IH and CM patients. Results: Abnormal BAEP were observed in 94% of IH patients, in 33% of CM and 70% of SNHL patients. After recovery from IH, BAEP abnormalities disappeared. Internal auditory canal (IAC)MRI abnormalities were described in 88% of IH patients. MRI signs of IH were observed in 33\u201378% in IH patients, but the most frequent MRI sign was 8th nerve T2 hyperintensity, with contrast enhancement in T1 sequences. This finding, combined with wave I latency, yielded highest specificity and sensitivity for IH diagnosis. Conclusions: Our study points out how IH can be effectively distinguished from CM and SNHL through the contribution of neurophysiology and MRI; in particular, evaluation of the 8th nerve achieves a high sensitivity and specificity in patients with IH. Further studies are required to examine the combined use of BAEP recordings ad MRI in diagnosis and monitoring of patients affected by IH

Inflammatory polyradiculoneuropathies: Clinical and immunological aspects, current therapies, and future perspectives

Inflammatory polyradiculoneuropathies are heterogeneous disorders characterized by immune-mediated leukocyte infiltration of peripheral nerves and nerve roots leading to demyelination or axonal degeneration or both. Inflammatory polyradiculoneuropathies can be divided into acute and chronic: Guillain–Barré syndrome and chronic inflammatory demyelinating polyneuropathy and their variants. Despite major advances in immunology and molecular biology have been made in the last years, the pathogenesis of these disorders is not completely understood. This review summarizes the current literature of the clinical features and pathogenic mechanisms of inflammatory polyradiculoneuropathies and focuses on current therapies and new potential treatment for the future

Posterior variant of alien limb syndrome with sudden clinical onset as self-hitting associated with thalamic stroke

We present a case of sudden postischaemic onset of alien limb syndrome, with unintentional self-injury. Alien limb syndrome is an uncommon neurological disorder featured by uncontrolled and involuntary movements of a limb. Three variants of alien limb syndrome have been described: the anterior, featured by grasping of surrounding objects, the callosal, presenting with intermanual conflict, and the posterior, associated with involuntary levitation of the limb. Our patient suffered from an acute presentation of the posterior variant of the alien limb syndrome, resulting from an isolated thalamic stroke which was documented using 24-h computed tomography brain scan. Only one previous case of alien limb syndrome after thalamic infarct has been reported. Our case enhances the possibility that pure thalamic injury may represent a trigger for this condition

Lateralized periodic discharges in insular status epilepticus: A case report of a periodic EEG pattern associated with ictal manifestation

Objective: Insular lobe seizures generally represent a misconceived ictal phenomenon characterized by specific neurological signs. Aphasia can be a rare presenting sign associated with insular lobe epilepsy which could be easily mistaken for a manifestation of other acute brain diseases. Method: We describe an insular status epilepticus (SE) characterized by sudden onset of language disturbance associated with hypersalivation and paraesthesia. A concomitant EEG recording showed the presence of Lateralized Periodic Discharges plus superimposed fast activity (LPDs + F). After an adequate acute endovenous anti-seizure treatment, a normalization of the EEG abnormalities with a complete resolution of all the neurological symptoms was achieved. Discussion: Language disturbances can be usually found in various pathological acute pictures involving the dominant frontal and temporal lobes. The presence of certain EEG pattern, could rise the suspect of aphasia as a critical manifestation. LPDs pattern is usually correlated with structural lesions. The association between LPDs and seizure is controversial but it seems to be more consistent when they are associated with “Plus modifiers” and with an high periodic frequency. Conclusion: Our case underlines the importance of considering focal SE in the differential diagnosis of patients presenting aphasia, even in the absence of previous history of epilepsy. We describe how LPDs can be associated with SE in a patient affected by a brain tumour, supporting the idea that some characteristic periodic patterns could be associated with seizure occurrence

The pharmacology of visual hallucinations in synucleinopathies

Visual hallucinations (VH) are commonly found in the course of synucleinopathies like Parkinson's disease and dementia with Lewy bodies. The incidence of VH in these conditions is so high that the absence of VH in the course of the disease should raise questions about the diagnosis. VH may take the form of early and simple phenomena or appear with late and complex presentations that include hallucinatory production and delusions. VH are an unmet treatment need. The review analyzes the past and recent hypotheses that are related to the underlying mechanisms of VH and then discusses their pharmacological modulation. Recent models for VH have been centered on the role played by the decoupling of the default mode network (DMN) when is released from the control of the fronto-parietal and salience networks. According to the proposed model, the process results in the perception of priors that are stored in the unconscious memory and the uncontrolled emergence of intrinsic narrative produced by the DMN. This DMN activity is triggered by the altered functioning of the thalamus and involves the dysregulated activity of the brain neurotransmitters. Historically, dopamine has been indicated as a major driver for the production of VH in synucleinopathies. In that context, nigrostriatal dysfunctions have been associated with the VH onset. The efficacy of antipsychotic compounds in VH treatment has further supported the notion of major involvement of dopamine in the production of the hallucinatory phenomena. However, more recent studies and growing evidence are also pointing toward an important role played by serotonergic and cholinergic dysfunctions. In that respect, in vivo and post-mortem studies have now proved that serotonergic impairment is often an early event in synucleinopathies. The prominent cholinergic impairment in DLB is also well established. Finally, glutamatergic and gamma aminobutyric acid (GABA)ergic modulations and changes in the overall balance between excitatory and inhibitory signaling are also contributing factors. The review provides an extensive overview of the pharmacology of VH and offers an up to date analysis of treatment options

Clinical presentation of strokes confined to the insula: a systematic review of literature

BACKGROUND AND PURPOSE: The insular cortex serves a wide variety of functions in humans, ranging from sensory and affective processing to high-level cognition. Hence, insular dysfunction may result in several different presentations. Ischemic strokes limited to the insular territory are rare and deserve a better characterization, to be quickly recognized and to receive the appropriate treatment (e.g. thrombolysis). METHODS: We reviewed studies on patients with a first-ever acute stroke restricted to the insula. We searched in the Medline database the keywords "insular stroke" and "insular infarction", to identify previously published cases. Afterwards, the results were divided depending on the specific insular region affected by the stroke: anterior insular cortex (AIC), posterior insular cortex (PIC) or total insula cortex (TIC). Finally, a review of the clinical correlates associated with each region was performed. RESULTS: We identified 25 reports including a total of 49 patients (59.7 ± 15.5 years, 48% male) from systematic review of the literature. The most common clinical phenotypes were motor and somatosensory deficits, dysarthria, aphasia and a vestibular-like syndrome. Atypical presentations were also common and included dysphagia, awareness deficits, gustatory disturbances, dysautonomia, neuropsychiatric or auditory disturbances and headache. CONCLUSIONS: The clinical presentation of insular strokes is heterogeneous; however, an insular stroke should be suspected when vestibular-like, somatosensory, speech or language disturbances are combined in the same patient. Further studies are needed to improve our understanding of more atypical presentations

A Critical Review of Alien Limb-Related Phenomena and Implications for Functional Magnetic Resonance Imaging Studies

Consensus criteria on corticobasal degeneration (CBD) include alien limb (AL) phenomena. However, the gist of the behavioral features of AL is still “a matter of debate.” CBD-related AL has so far included the description of involuntary movements, frontal release phenomena (frontal AL), or asomatognosia (posterior or “real” AL). In this context, the most frequent symptoms are language and praxis deficits and cortical sensory misperception. However, asomatognosia requires, by definition, intact perception and cognition. Thus, to make a proper diagnosis of AL in the context of CBD, cognitive and language dysfunctions must be carefully verified and objectively assessed. We reviewed the current literature on AL in CBD and now propose that the generic use of the term AL should be avoided. This catchall AL term should instead be deconstructed. We propose that the term AL is appropriate to describe clinical features associated with specific brain lesions. More discrete sets of regionally bound clinical signs that depend on dysfunctions of specific brain areas need to be assessed and presented when posing the diagnosis. Thus, in our opinion, the AL term should be employed in association with precise descriptions of the accompanying involuntary movements, sensory misperceptions, agnosia-asomatognosia contents, and the presence of utilization behavior. The review also offers an overview of functional magnetic resonance imaging-based studies evaluating AL-related phenomena. In addition, we provide a complementary set of video clips depicting CBD-related involuntary movements that should not mistakenly be interpreted as signs of AL

Data of safety in a single-center alemtuzumab treated population

Alemtuzumab is approved for highly active MS and, in Europe, can be employed after other disease-modifying treatments (DMTs) as an escalation approach or first therapeutic option. The occurrence of secondary autoimmune adverse events and infections differs depending on the employed approach. In the manuscript entitled “Alemtuzumab treatment of multiple sclerosis in real-world clinical practice: report from a single Italian center” by di Ioia M. and collaborators, efficacy and safety data of alemtuzumab were evaluated in a real-world MS population. The aim of the article is to describe in detail the unexpected serious adverse events which occurred in this cohort during and after the administration of the alemtuzumab treatment. Adverse events were observed in 45,7% of the patients. These events were ranked as severe in 23% of the patients. We reported, in particular, cases of autoimmune hemolytic anemia (AIHA), pancytopenia, viral hepatitis E and noninfectious meningo-encephalomyelitis

Neurology and the COVID-19 emergency

Following the COVID-19 outbreak, significant changes have been implemented on a national level in the organization of neurology units and associated stroke units. Regionallydesignated COVID-19 hospitals have implemented an aggressive policy to relocate as many beds as possible to COVID-19 patients. In order to do so, the preferred strategy has been to reduce the number of beds in neurology units, and in some cases several units have been consolidated into one. In other cases, particularly in the northern regions