Focal neurological deficit in acute anemia: Case reports and review of the literature

Introduction: Although anemia is not regarded as an usual vascular risk factor for stroke, it is one of the potential mechanism by which the brain does not receive adequate oxygenation. Moreover, the relationship between drop of hemoglobin and acute focal neurological deficits is not clear. We report two patients with cerebral infarction due to acute anemia. Case Reports: Case 1 was a 73-year-old man who complained an episode of loss of consciousness followed by right hemiparesis and dysarthria after few hours. The day after admission he presented melena caused by a duodenal ulcer bleeding. The hemoglobin dropped from 11.3 g/dl to5.6 g/dl in 24 hours. Areas of acute infarctions were evident at diffusion-weighted imaging (DWI) of the brain. Case 2 was a 77-year-old man with a transient episode of aphasia, right lower limb paresis and mental confusion twelve hours after an intervention of PTA and stenting of the left internal carotid artery. Hemoglobin was11.8 g/dl before intervention and9 g/dl 48 hours later. DWI showed bilateral and widespread acute infarcts. Conclusion: Anemia has to be considered as a potential factor in determining or worsening cerebral infarction, especially in patients with carotid or intracranial stenosis, high cerebrovascular lesions load or insufficient collateral supply. Acute or severe anemia may negatively impact the cerebral blood flow and decrease oxygen-carrying capacity, promote rapid deterioration of ischemic penumbra. Brain DWI and treatment of the underlying etiology of acute anemia are crucial in early identification and recovery of cerebral infarctions.

Impact of COVID-19 pandemic on the neuropsychiatric status of Wilson’s disease

We have read with interest the Letter to the Editor by Drs.Zhuang and Zhong,who presented the clinical data of 68 patients with Wilson’s disease(WD)who were admitted to the hospital before and during the coronavirus disease 2019(COVID-19)pandemic,and appreciated their findings on hepatic and some extrahepatic manifestations.Nevertheless,given the strong impact of the pandemic on patients with neurological and psychiatric disorders,we would have expected a worsening of the psychiatric and/or neurological impairments in these patients.In contrast,according to the authors,these manifestations remained,somewhat unexpectedly,unchanged.This finding is in contrast with most of the current literature that highlights not only an increased incidence of mental health disorders in the general population but also an exacerbation of neurological and psychiatric symptoms in patients with chronic diseases,especially in those with pre-existing neuropsychiatric disorders,such as WD.Although the study was mainly focused on the hepatic features of WD patients taking anti-copper treatment,a generic and cumulative definition of neurological and psychiatric manifestations,as in this study,does not allow for further considerations.Future studies during and after the pandemic are necessary to clarify the real impact,either direct or indirect,of the COVID-19 pandemic on the neurological and psychiatric symptoms of WD patients.

Repetitive transcranial magnetic stimulation for post-traumatic stress disorder:Lights and shadows

We have read with interest the publication that describes the available data related to the use of neuromodulation strategies for the treatment of posttraumatic stress disorder(PTSD).Despite treatment advances,however,a substantial proportion of PTSD patients receiving psychological and/or pharmacological treatment do not reach an adequate clinical response.In their paper,the authors draw attention to the current understanding of the use of repetitive transcranial magnetic stimulation(rTMS)as a potential treatment for PTSD.Most of the previous studies indeed applied both inhibitory(1 Hz)and excitatory(>1 Hz,up to 20 Hz)rTMS to the right and/or left dorsolateral prefrontal cortex.Despite larger therapeutic effects observed when high-frequency stimulation was applied,the question of which side and frequency of stimulation is the most successful is still debated.The authors also reported on the after-effect of rTMS related to neuroplasticity and identified the intermittent theta burst stimulation as a technique of particular interest because of it showed the most effective improvement on PTSD symptoms.However,although numerous studies have highlighted the possible beneficial use of rTMS protocols for PTSD,the exact mechanism of action remains unclear.In their conclusions,the authors stated that rTMS has been demonstrated to be effective for the treatment of PTSD symptoms.Nevertheless,we believe that further research with homogeneous samples,standardized protocols,and objective outcome measures is needed to identify specific therapeutic targets and to better define significant changes when active and sham stimulation procedures are compared.

Course of Atypical Manifestations of a Case of Charcot-Marie-Tooth Disease over 35 Years of Clinical Observation

Introduction: Atypical manifestations of peripheral neuropathy are not rare, challenging the differential diagnosis. In the past, the diagnosis of hereditary neuropathy was mainly based on the clinical and electromyographic (EMG) findings and, occasionally, biopsy. Nowadays, the genetic tests allow us to identify more than 40 different genes/loci associated with Charcot-Marie-Tooth (CMT) disease, although some subtypes are clinically indistinguishable. We have followed a patient with a clinical diagnosis of apparent sporadic and atypical CMT and recently diagnosed genetically as distal hereditary motor neuropathy, type V (dHMN-V). Case Report: Thirty-five years ago, a 16 years old patient complained muscular weakness and wasting at the hands small muscles. Sporadic fasciculations were observed whereas deep tendon reflexes and sensation were normal. EMG examination revealed neurogenic muscular denervation in the distribution of C7, C8 and T1 segments bilaterally. Muscular biopsy of the left Biceps Brachii showed rare atrophic fibers and some cellular atypia. The disease has undergone a clinical and EMG progression and diffusion over the years, involving the lower limbs and leading to a bilateral steppage. A more slight diffuse axonal motor neuropathy was also identified in the proband’s son and second cousin. The genetic study found a known missense mutation in BSCL2 gene related to a dHMN-V. Interestingly, there was a remarkable intra-familiar phenotypic variability, especially in the clinical onset and severity. Discussion: Atypical manifestations of hereditary neuropathies often overlap with other conditions. The present case highlights how a comprehensive clinical evaluation and a careful follow-up have led to a correct diagnosis even 35 years later and have allowed to identify other affected family members. The apparent lack of familiarity was probably due to the very soft presentation in the proband’s relatives. Although the genetic study was not available at that time, the first clinical diagnosis was not disavowed. The main differential diagnoses and a brief review of similar reported cases are discussed.