Elia Sechi

Anatomical variability of the lateral femoral cutaneous nerve: findings from a surgical series

The lateral femoral cutaneous nerve (LFCN) is a branch of the lumbar plexus and supplies the skin of the lateral thigh region. This entrapment‐compressive syndrome is named meralgia paresthetica or Roths meralgia and depends, on a vast majority of cases, on the entrapment of the nerve in proximity of the inguinal ligament. Surgical decompression of the nerve is an option when conservative treatments fail and is usually performed through a 3‐cm infrainguinal skin incision. Available data on anatomical variations of the LFCN derive from extensive cadaver dissections and lack many features relevant to the surgeon. This study was conducted to investigate anatomical details of the LFCN at the site of surgery for meralgia paresthetica. We reviewed retrospective data regarding the anatomical features of LFCN from 148 consecutive patients operated on for Roths meralgia. In the majority of the cases the LFCN was a single trunk, deep to the thigh superficial fascia and to the inguinal ligament and coursing inferior‐lateral to the anterior superior iliac spine. Less frequent findings were early nerve bifurcation, epifascial position, inferior‐medial direction, and exit from the pelvis through an iliac bone canal. In 13 cases (8.8%) the nerve was not found at surgery. Anatomical variations of the LFCN must be considered at the time of surgery to maximize success rates and avoid nerve damage during surgical dissection. Clin. Anat. 22:365–370, 2009.

Clinical spectrum and IgG subclass analysis of anti-myelin oligodendrocyte glycoprotein antibody-associated syndromes: a multicenter study

Anti-myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) recently emerged as a potential biomarker in patients with inflammatory demyelinating diseases of the central nervous system. We here compare the clinical and laboratory findings observed in a cohort of MOG-Ab seropositive and seronegative cases and describe IgG subclass analysis results. Consecutive serum samples referred to Verona University Neuropathology Laboratory for aquaporin-4 (AQP4)-Ab and/or MOG-Ab testing were analysed between March 2014 and May 2017. The presence of AQP4-Ab was determined using a cell-based assay. A live cell immunofluorescence assay was used for the detection of MOG-IgG and IgG subclass analysis. Among 454 analysed samples, 29 were excluded due to AQP4-Ab positivity or to the final demonstration of a disorder not compatible with MOG-Ab. We obtained clinical data in 154 out of 425 cases. Of these, 22 subjects resulted MOG-Ab positive. MOG-Ab positive patients were mainly characterised by the involvement of the optic nerve and/or spinal cord. Half of the cases presented relapses and the recovery was usually partial. Brain MRI was heterogeneous while short lesions were the prevalent observation on spinal cord MRI. MOG-Ab titre usually decreased in non-relapsing cases. In all MOG-IgG positive cases, we observed IgG1 antibodies, which were predominant in most subjects. IgG2 (5/22), IgG3 (9/22) and IgG4 (3/22) antibodies were also detectable. We confirm that MOG-Ab-related syndromes have distinct features in the spectrum of demyelinating conditions, and we describe the possible role of the different IgG subclasses in this condition.

Ceftriaxone for Alexander’s Disease: A Four-Year Follow-Up

In 2010, we reported the successful clinical outcome related to a 20-month course of intravenous, cyclical ceftriaxone, in a patient with adult-onset Alexanders disease. We now provide evidence that the progression of the patients signs/symptoms was halted and reversed with a 4-year-long extension of the trial.The patients clinical signs/symptoms were evaluated before the start and every 6 months for 6 years. For the early 2 years, without therapy, and for the following 4 years, after intravenous ceftriaxone 2 g daily, for 3 weeks monthly during the initial 4 months, then for 15 days monthly.Gait ataxia and dysarthria were assessed clinically on a 0 to 4 scale. Palatal myoclonus and nystagmus/oscillopsia were monitored by videotape and a self-evaluation scale. The degree of disability, measured by a modified Rankin scale, and the brain MRI were periodically evaluated.Before ceftriaxone therapy, in a 2-year period, gait ataxia and dysarthria worsened from mild to marked, palatal myoclonus spread from the soft palate to lower facial muscles, and the patient complained of oscillopsia. After 4 years of ceftriaxone therapy, gait ataxia and dysarthria improved, from marked to mild at clinical rating scales. The palatal myoclonus was undetectable; the patient did not complained of oscillopsia and declared a progressively better quality of life. Ceftriaxone was safe.This case report provides Class IV evidence that intravenous cycles of ceftriaxone may halt and/or reverse the progression of neurodegeneration in patients with adult-onset Alexanders disease and may significantly improve their quality of life.

Teaching NeuroImages: Subacute encephalopathy in a young woman with THTR2 gene mutation

A 21-year-old woman presented with coma after 5 days of fever, gait ataxia, and somnolence. Brain MRI showed lesions in medial thalami, caudate heads, and periaqueductal region (figure). Hyperlactatemia was present; serum thiamine levels were normal.

Late presentation of NMOSD as rapidly progressive leukoencephalopathy with atypical clinical and radiological findings

Brain abnormalities in neuromyelitis optica spectrum disorder (NMOSD) are highly heterogeneous and often non-specific. Extensive white matter involvement has been described and frequently manifests with encephalopathy requiring prompt intervention. Rarely, this may represent the only manifestation at onset without concurrent suggestive features of the disease, thus making diagnosis challenging. NMOSD may potentially occur at any age, but it seems that this disorder has distinctive clinical features in the elderly. We describe a case of NMOSD presenting as rapidly progressive leukoencephalopathy with atypical clinical and magnetic resonance imaging (MRI) findings in a 69-year-old woman.

Mycobacterium avium subspecies paratuberculosis and myelin basic protein specific epitopes are highly recognized by sera from patients with Neuromyelitis optica spectrum disorder

Epstein-Barr virus (EBV) is the main environmental agent associated to neuromyelitis optica spectrum disorder (NMOSD). Following to studies reporting an increased prevalence of antibodies against peptides derived from Mycobacterium avium subsp. paratuberculosis (MAP) homologous to EBV and human epitopes (MBP85-98, IRF5424-434) in multiple sclerosis (MS), we investigated whether seroreactivity to these antigens display a NMOSD-specific pattern. The sera of 34 NMOSD patients showed elevated levels of antibodies against MAP and MBP compared to healthy controls (44% vs. 5%, p < 0.0002 and 50% vs. 2%, p < 0.0001, respectively), while, unlike in MS, responsiveness to EBV was similar.

Restless legs syndrome and cerebrovascular disease

734 www.thelancet.com/neurology Vol 12 August 2013 Restless legs syndrome and cerebrovascular disease We read with interest Raja Mehanna and Joseph Jankovic’s Review about movement disorders in patients with cerebrovascular disease. We were surprised that in this well planned review restless legs syndrome (RLS) was completely overlooked. RLS is a common neurological movement disorder characterised by a compelling urge to move the legs, usually accompanied by uncomfortable and unpleasant sensations deep in the legs, which begins or worsens during periods of rest or inactivity, and is partly or totally relieved by movement. The prevalence of RLS in the general population is 3–10%, which makes it the most common movement disorder. Women are twice as likely to be aff ected as men, and prevalence has been shown to increase with age. RLS is still greatly underdiagnosed. It can be idiopathic or secondary to several conditions including focal cerebrovascular disease. In particular, the prevalence of post-stroke RLS is about 12·4%, with a topography of lesions that mainly aff ect subcortical brain areas such as the pyramidal tract and the basal ganglia-brainstem axis, which are involved in motor functions and sleep-wake cycles. Post-stroke RLS is often associated with periodic limb movements of sleep (PLMS). The pathophysiology of RLS is still unclear. The discrete brain localisations seen in patients with post-stroke RLS suggest a possible role for specifi c structures in the lenticulostriate region. A lesion in this area (nuclear complex or fi bre systems) might exert both an ascending disinhibition on the sensorimotor cortex and a disinhibition of descending inhibitory pathways, which could result in a facilitation of RLS and PLMS. As in idiopathic RLS, in post-stroke RLS dopamine agonists such as pramipexole can be benefi cial, which suggests that dopamine hypoactivity might have a pathophysiological role. We declare that we have no confl icts of interest.

Antibody response against HERV-W env surface peptides differentiates multiple sclerosis and neuromyelitis optica spectrum disorder

Background A specific humoral immune response against HERV-W envelope surface (env-su) glycoprotein antigens has been reported in serum of patients with multiple sclerosis (MS). However, it has not been evaluated to date in patients with neuromyelitis optica spectrum disorder (NMOSD). Objective The objective of this paper is to investigate whether antibody (Ab) response against HERV-W env-su antigenic peptides differs between NMOSD and MS. Methods Serum samples were collected from 36 patients with NMOSD, 36 patients with MS and 36 healthy control individuals (HCs). An indirect ELISA was set up to detect specific Abs against HERV-W env-su peptides. Results Our data showed that two antigenic peptides, particularly HERV-Wenv93–108 and HERV-Wenv248–262, were statistically significantly present only in serum of MS compared to NMOSD and HCs. Thus, the specific humoral immune response against HERV-W env-su glycoprotein antigens found in MS is widely missing in NMOSD. Conclusion Increased circulating serum levels of these HERV-W Abs may be suitable as additional biomarkers to better differentiate MS from NMOSD.

Neuralgic amyotrophy mimicking Vernet syndrome

A 72-year-old Caucasian man, with a medical history of arterial hypertension, was admitted in our Neurology Department for acute pharyngodynia and extreme pain localized to the neck, right scalp and the ipsilateral ear canal, followed two days later by dysphagia and hoarseness. One week before he had suffered from a flu-like illness with fever. The pain resolved in 5 dayswhereas hoarseness and dysphagia persisted. At laryngoscopic examination right vocal-cord palsy was noted (Fig. 1). Neurological examination showed weakness of the right soft-palate with reduced gag-reflex and right shoulder ptosis with weakness (4/5 MRC) of right sternocleidomastoid and trapezius muscles. No fasciculation or scapular winging were noted. Sensory examination was unremarkable. Examination of the mouth and external auditory canal did not show any vescicular rash. Considering the unilateral involvement of IX, X and XI cranial nerves, the common causes of Vernet syndrome were investigated. Laboratory tests including blood count, serum-protein electrophoresis, immunofixation, C-reactive protein, creatine kinase, erythrocyte-sedimentation rate, TSH, FT3, FT4, antinuclear antibodies (ANA), extractable-nuclearantigens antibodies, antineutrophil-cytoplasmatic antibodies (ANCA), rheumatoid factor, antiganglioside antibodies, anti-AchR and antiMUSK antibodies, Lyme test, syphilis, varicella-zooster virus (VZV), Epstein–Barr virus (EBV), hepatitis A, B and C, and human immunodeficiency virus serologies (HIV) were normal. Cerebrospinal fluid analysis did not show any hyperproteinorrachia, pleocytosis or oligoclonal bands. PCR did not detect VZV or herpes simplex virus. Chest X-ray was negative. Brain and neck MR studies, CT scan of the basicranial region, and carotid ultrasonography failed to identify anymass or infiltrative lesion along the course of IX, X and XI cranial nerves. Nerve conduction studies and F-wave were normal. Electromyography exhibited fibrillation potentials and positive sharp-waves in the right trapezium, right sternocleidomastoid, deltoid muscles bilaterally, and in the left infraspinatus muscle. No abnormalities were noted in the cervical paraspinal muscles. Motor unit potentials were of normal configuration with reduced recruitment. Brachial plexus MR was normal. On the basis of the clinical history of intense pain followed two days later by weakness (dysphagia, hoarseness, right shoulder ptosis), considering the electromyographic pattern of mononeuritis multiplex and

Clinicopathologic features of folate-deficiency neuropathyAuthor Response

Koike et al.1 reported a slowly progressive, prevalent sensory polyneuropathy in patients with folate deficiency, which shows some distinctive clinical features with respect to thiamine-deficiency neuropathy.2 However, as the authors mention, the clinical manifestations of folate deficiency may be indistinguishable from those of cobalamin deficiency because of their interaction in one carbon metabolic pathway.3 In addition, thiamine is poorly absorbed during folate deficiency, because of frequent diarrhea as a result of damaged gastrointestinal mucosa. …