Upper and Lower Face and Ideomotor Apraxia in Patients with Alzheimer’s Disease

Introduction: Apraxia of face movement in Alzheimer's disease (AD) has been rarely investigated. This study aimed at investigating the frequency of lower (mouth, tongue and throat) and upper (eyes and eyebrows) face apraxia, in AD and its relationship with limb apraxia and severity of dementia

Is there a decrease in Guillain-Barré syndrome incidence after bovine ganglioside withdrawal in Italy? A population-based study in the Local Health District of Ferrara, Italy.

There have been many reports of cases of Guillain-Barré syndrome (GBS) after therapeutic injection of bovine ganglioside preparations with the result that they were withdrawn in Italy in December 1993. As the relationship between bovine gangliosides and GBS has not yet been established, a further epidemiological investigation in the Local Health District (LHD) of Ferrara, Italy, was carried out in the years 1994-2001 to verify whether the incidence of GBS had changed after ganglioside withdrawal. The other aim of this investigation was to update the incidence of GBS in this area since the two previous investigations we carried out showed an increase in incidence from the years 1981-1987 to the years 1988-1993. The cases of GBS were identified prospectively. To guarantee completeness of case ascertainment, an intensive retrospective survey of all possible sources of cases for the entire study period was performed. The mean annual crude incidence rate in the years 1994-2001 (based on 26 new cases) was 1.97 per 100,000 population (95% CI 1.29-2.89), whereas it had been 1.87 per 100,000 population (95% CI 1.35-2.52) in the years 1981-1993 (based on 43 cases) when gangliosides were available. The age-adjusted rates were almost identical (1.66 and 1.65 per 100,000 population, respectively). Although ganglioside administration could have triggered, on the basis of an individual susceptibility, an immunologic reaction which produced GBS, the incidence of GBS in the study area did not change after ganglioside withdrawal. In the whole period 1981-2001, a temporal pattern of incidence was reported with an increase towards a peak in 1990-1992 and a progressive decline thereafter. This temporal pattern did not seem related to ganglioside withdrawal, and no definite explanation for it was found which could imply that the disease incidence is less stable than it was deemed

A trial to prove the efficacy of acupuncture as a therapeutic support in pharmacological prophylaxis for migraine and tension-type headache. Pilot study.

Importance: the effectiveness of acupunture as a therapeutic support in pharmacological prophilaxis for migraine and tension-type headache. Objective: to define the role of acupuncture in the treatment and prophylaxis of various types of migraine by measuring the effects it produces in preventing the disease, reducing symptoms and their prolongation over time. Design, setting and partecipants: this work, which may be considered a pilot study, provides the clinical results obtained in a period of four months from September 2018 to September 2019. The 42 participants, recruited from patients of the Headache Center of the Neurology Unit of Sant'Anna University Hospital of Ferrara, were randomly divided into two groups. Group A received pharmacological prophylaxis (Control group), while Group B undertook pharmacological prophylaxis and acupuncture (Experimental group). Interventions: The control group took medication for four months, while the experimental group received a twice-weekly treatment of eight-twenty minutes acupuncture sessions every two weeks. Main outcome and measures: all participants were examined before the start of prophylaxis (T0), after two months (T1) and after four months (T2). They were asked to evaluate the daily progress of their psycho-physical conditions in a diary, from which it emerged that the practice of acupuncture reduced not only the frequency and intensity of daily attacks, but also the use of analgesics, revealing a prolongation of the positive effects for four months. Results: Group B showed a significant reduction in the Henry Ford Headache Disability Inventory - beta (β-HDI) score in both the second and fourth month compared to Group A, with a similar pattern emerging for the Migraine Disability Assessment Score (MIDAS). Likewise, an improvement in physical conditions and a decrease in pain were recorded as far as the Short Form 36 Health Survey (SF-36) scale was concerned. Conclusions and relevance: this study shows that acupuncture is a valid supportive therapy for pharmacological prophylaxis of migraine and tension-type headache

Idiopathic cervical dystonia and non-motor symptoms: a pilot case-control study on autonomic nervous system

Purpose: Non-motor symptoms, such as sleep disturbances, fatigue, neuropsychiatric manifestations, cognitive impairment, and sensory abnormalities, have been widely reported in patients with idiopathic cervical dystonia (ICD). This study aimed to clarify the autonomic nervous system (ANS) involvement in ICD patients, which is still unclear in the literature. Methods: We conducted a pilot case-control study to investigate ANS in twenty ICD patients and twenty age-sex-matched controls. The Composite Autonomic System Scale 31 was used for ANS clinical assessment. The laser Doppler flowmetry quantitative spectral analysis, applied to the skin and recorded from indices, was used to measure at rest, after a parasympathetic activation (six deep breathing) and two sympathetic stimuli (isometric handgrip and mental calculation), the power of high-frequency and low-frequency oscillations, and the low-frequency/high-frequency ratio. Results: ICD patients manifested higher clinical dysautonomic symptoms than controls (p < 0.05). At rest, a lower high-frequency power band was detected among ICD patients than controls, reaching a statistically significant difference in the age group of ≥ 57-year-olds (p < 0.05). In the latter age group, ICD patients showed a lower low-frequency/high-frequency ratio than controls at rest (p < 0.05) and after mental calculation (p < 0.05). Regardless of age, during handgrip, ICD patients showed (i) lower low-frequency/high-frequency ratio (p < 0.05), (ii) similar increase of the low-frequency oscillatory component compared to controls, and (iii) stable high-frequency oscillatory component, which conversely decreased in controls. No differences between the two groups were detected during deep breathing. Conclusion: ICD patients showed ANS dysfunction at clinical and neurophysiological levels, reflecting an abnormal parasympathetic-sympathetic interaction likely related to abnormal neck posture and neurotransmitter alterations

Acute Painful Neuropathy in a Heroin Body Packer

A broad range of peripheral neurological complications have been recognized in heroin addicts. Peripheral Nervous System (PNS) involvement, usually secondary to trauma during loss of consciousness, commonly manifests as compressive neuropathy or rhabdomyolysis. Other, rare, non traumatic PNS lesions include poliradiculopathy, plexopathy, Guillan-Barrè syndrome and mononeuropathy. Acute heroin related non compressive neuropathy is rare, and the aetiology is still unclear. In heroin abusers painful acute neuropathy has rarely been described. We report the case of a 33-year-old male with a history of heroin abuse. He was found in his home in coma, maintaining the same sitting position for several hours. He was admitted to the intensive care unit, where rhabdomyolysis complicated by acute renal failure was diagnosed, requiring treatment with haemodialysis. A Computed Tomography (CT) scan of the abdomen revealed the presence of several foreign bodies in sigma and the urinary exams showed high concentration of opiates, revealing that the patient was a body packer. Further laboratory exams showed Hepatitis C Virus (HCV) infection with normal liver function. Upon awakening, five days after admission, the patient complained of severe burning pain, mechanical allodynia, itch, tingling and pinprick sensation in both feet and legs. The Douleur Neuropathique en 4 Questions (DN4) scale was 8/10, Numeric Rating Scale (NRS) scale score was 9/10 and Neuropathic Pain Symptom Inventory (NPSI) score was 37. Neurologic examination showed reduced sensitivity to temperature and pinprick in both legs and feet. Neurophysiological evaluation, performed 4 weeks after admission, showed normal nerve conduction studies. Thermal Quantitative Sensory Testing (t-QST) showed a patchy distribution of cold and warm hypoesthesia in the lower limbs. The patient also described positive symptoms (hyperalgesia and aftersensation following hot stimuli, mechanical static and dynamic allodynia) in the same areas. Sympathetic Skin Response (SSR) performed in both arms and feet was normal. Laser Doppler Flowmetry showed reduced local vasodilation after heat stimulus (C-mediated local axonal reflex) and normal vasoconstriction reflex (mediated by adrenergic sympathetic fibres) distally in the legs. The patient declined undergoing a skin biopsy for diagnostic purposes. The clinical and neurophysiological findings indicate the presence of small fibre neuropathy, with main involvement of C fibres, following a length-dependent distribution. To relieve the severe painful symptoms the patient was treated with gabapentin, fentanyl, duloxetina and pregabalin, with a gradual resolution of pain. The follow up examination, performed after 12 months, showed an improvement of the small fibre related symptoms with resolution of spontaneous pain and attenuation of itch, tingling and pinprick sensation, confirmed by the t-QST evaluation. A broad range of peripheral neurological complications have been recognized in heroin abusers. Peripheral nerve lesions in drug addicts may be caused by injection of substances in the vicinity of a nerve, local infection or nerve compression during coma. Combined nerve or plexus lesions and rhabdomyolysis have been reported in relation to possible trauma but they may occur also without apparent trauma to muscles or nerves. In these latter cases toxic or allergic reaction to heroin or adulterants are probably more important causes of rhabdomyolysis and nerve lesions than limb compression. Reports on acute heroin-related non compressive neuropathy are scarce. Dabby et al. described six patients who developed acute PNS injury following intravenous or intranasal heroin self administration with no evidence of compression injury or inflammation. Four patients had plexopathy and two had symmetric distal axonal sensorimotor neuropathy; five had concomitant rhabdomyolysis. They proposed that a toxic mechanism could be responsible for acute neuropathy following heroin abuse. In heroin abusers painful complications have been reported, but the pain was related to myelopathy, plexopathy, sciatic neuropathy and rhabdomyolysis4; none of these cases had length-dependent symptoms or clinical signs of small fibre damage. Numerous studies in humans and animals report that opioids can elicit abnormal pain, like thermal hyperalgesia and/or mechanical allodynia, following both acute and chronic administration of morphine, heroin, methadone, fentanyl and remifentanil. The mechanism responsible for the tactile and thermal hypersensitivity observed following opioid exposure is still unclear. Many hypotheses have been put forward, including sensitization of peripheral nerve endings or second-order neurons, enhanced descending facilitation of nociceptive pathways, increased production, release and decreased re-uptake of neurotransmitters involved in nociception, activation of glial cells and opioid associated epigenetic changes in Deoxyribonucleic Acid (DNA) methylation. Prolonged exposure to opioids results in long-lasting neuroadaptive changes that promote a state of hypersensitivity to normal non-noxious tactile and to noxious thermal stimulation that may promote activation of the nociceptive system. The aetiology of acute PNS complication in heroin addicts can be related to different mechanisms. The impurities and additives present in illicit drugs may cause allergies and have their own toxic effects; mechanical trauma is a potential mechanism of focal nerve injury and rhabdomyolysis. Evidence of an immunologic cause has been found in three patients who developed acute brachial plexopathy and rhabdomyolysis after heroin administration and in one heroin addict who developed a lumbosacral plexus neuropathy and responded to steroid therapy. We describe the case of a body packer who developed acute painful neuropathy with involvement of small nerve fibre with concomitant rhabdomyolysis and acute renal failure. The patient was HCV positive; all the other causes of Small Fibre Neuropathy (SFN) were excluded. In our patient there was no evidence of an inflammatory or immunologic process and mechanical factors probably didn’t play a major role in the pathogenesis. The patient had a history of prolonged lying before arriving to the emergency room but the neuropathy was symmetric and distal, being inconsistent with focal trauma or compression. Acute renal failure and haemodialysis can be possible causes of SFN but in our patient there was not a correlation of the symptoms with the haemodialytic sessions and there was not an improvement of symptoms after achieving normal renal function. HCV infection can play a role in SFN, but the acute presentation of the symptoms in temporal relation with heroin intoxication and their gradual improvement suggest that a toxic effect of heroin or added substances can be responsible for neural and muscle toxicity in our patient. In conclusion a careful neurophysiological evaluation should be considered in heroin abusers presenting with acute pain to rule out SFN

La cefalea nella malattia di Behçet: contributo casistico e revisione della letteratura.

Objective: To evaluate the prevalence of headache and its different patterns in patients with Behçet’s disease (BD) with and without neurological involvement and to investigate clinical correlations. Methods: Patients fulfilling the International Study Group criteria for Behçet disease (ISGc) were studied. Patients were invited to fill a “headache questionnaire”, which consisted of two sections: the first one included demographic and anamnestic data, family history for both headache and BD, disease duration and clinical manifestations of BD; the second section included items about headache, investigated accordingly to International Headache Society diagnostic criteria (IHS, 2004). Clinical history and current comorbidities-medications were collected. Each patient underwent a neurological examination to assess neurological involvement (Neuro-BD) and, if necessary, instrumental investigations. One hundred-fifty healthy subjects matched for age and gender were used as control group for comparison. Results: Of the 55 patients diagnosed as BD (ISG criteria) 41 patients adhered and were enrolled into the study. Headache occurred in 29 of BD patients (70,7%) and in 13 of Neuro-BD patients (92,8%). Migraine without aura did prove the most frequent type of headache in BD patients (with and without neurological involvement) and there were no differences in the frequency of the different pattern of headache between BD patients and controls. Conclusions: Headache is a frequent manifestation in BD and primary headache like migraine emerged as the most frequent type of headache. A careful search for headache should be ncluded in the diagnostic work-up of BD since this manifestation may be related to the underlying disease

Intraoperative monitoring of sensory part of the trigeminal nerve using blink reflex during microvascular decompression for trigeminal neuralgia.

Intraoperative monitoring during cerebellopontine angle surgery is widely accepted. While techniques which monitor cranial motor nerves are commonly used, monitoring the sensory afferents has been challenging. Considering the reflex arc, blink reflex (BR) might be useful in monitoring the sensory part of the trigeminal nerve, the brainstem connections and the facial nerve. We describe the case of a patient who developed hemifacial hypoesthesia after microvascular decompression surgery for trigeminal neuralgia. Intraoperative BR showed a severe loss of R1 amplitude. BR might be a useful intraoperative technique to monitor the sensory part of the trigeminal nerve

Dental amalgam and multiple sclerosis: a case-control study in Ferrara, Italy

Background: Epidemiological data on Multiple Sclerosis (MS) have enphasized the role of both genetic and environmental factors in the etiology. Despite the weakness of biological plausibility, dental amalgams containing mercury have been suggested as a possible risk factor. Methods: In a community-based case-control study carried out in the Province of Ferrara, we interviewed 132 MS cases with diseases onset during the last 10 years and 423 controls, to obtain information on socio-demographic characteristics and the number of dental amalgams and the time since their installation. Results: Odds ratios (OR) for subjects with exposures of different duration and with different numbers of amalgams were not statistically significant. The comparison between subjects with no fillings and those with protracted exposures to many amalgams did not give significant results. The group with exposure before 15 years of age did not seem to have larger OR than those with exposure beginning later in life. Conclusions: Neither the number nor the duration of exposure to amalgams supported the hypothesis of an increased risk ofMS, in agreement with another study carried out in Canada and addressing the same topic

The Incidence of Myasthenia Gravis in the Province of Ferrara, Italy, in the Period of 2008–2022: An Update on a 40-Year Observation and the Influence of the COVID-19 Pandemic

: Myasthenia gravis (MG) is the most common neuromuscular junction disorder. We evaluated the MG incidence rate in the province of Ferrara, Northern Italy, over two time frames (2008-2018 and 2019-2022, i.e., the COVID-19 pandemic) and considered early-onset (EOMG), late-onset (LOMG), and thymoma- and non-thymoma-associated MG. Moreover, in the second period, we assessed its possible relationship with SARS-CoV-2 infection or COVID-19 vaccination. We used a complete enumeration approach to estimate the MG incidence and its temporal trend. For the period of 2008-18, 106 new cases were identified (mean incidence rate 2.7/100,000 people). The highest rates were observed for the over-70 age group and in rural areas, with 17% of thymoma-associated MG. During the COVID-19 period, 29 new cases were identified (average incidence rate 2.1/100,000 people), showing a marked (though not statistically significant) decrease in the mean annual incidence compared to the previous period. Again, the highest rate was observed for the over-70 age group. The first period was in line with our previous observations for the period between 1985 and 2007, highlighting a rising incidence of LOMG and a marked decrease in EOMG. During the COVID-19 period, incidence rates were lower in the first years whereas, when the pandemic ended, the previous trend was confirmed