Diagnostic accuracy of laser-evoked potentials in diabetic neuropathy.

Although the most widely agreed neurophysiological tool for investigating small fiber damage is laser-evoked potential (LEP) recording, no study has documented its diagnostic accuracy. In this clinical, neurophysiological, and skin biopsy study, we collected age-corrected LEP normative ranges, verified the association of LEPs with pinprick sensory disturbances in the typical diabetic mixed fiber polyneuropathy, and assessed the sensitivity and specificity of LEPs in diabetic small fiber neuropathy. From 288 LEP recordings from the face, hand, and foot in 73 healthy subjects, we collected age-corrected normative ranges for LEPs.

Laryngeal Sensitivity in Patients with Amyotrophic Lateral Sclerosis.

Recent studies have shown the involvement of the sensory nervous system in patients with amyotrophic lateral sclerosis (ALS). The aim of our study was to investigate the correlation between the laryngeal sensitivity deficit and the type of ALS onset (bulbar or spinal) in a large series of 114 consecutive ALS patients. Participants were subdivided into two groups, bulbar and spinal ALS, according to the clinical onset of disease and submitted to a clinical and instrumental evaluation of swallowing, including a fiber-optic endoscopic evaluation of swallowing with sensory testing.

A Novel Mutation in Gene Causing Tangier Disease in an Italian Family with Uncommon Neurological Presentation.

Tangier disease is an autosomal recessive disorder characterized by severe reduction in high-density lipoprotein cholesterol and peripheral lipid storage. We describe a family with c.5094C > A p.

Ion channels expression and function are strongly modified in solid tumors and vascular malformations.

Background: Several cellular functions relate to ion-channels activity. Physiologically relevant chains of events leading to angiogenesis, cell cycle and different forms of cell death, require transmembrane voltage control. We hypothesized that the unordered angiogenesis occurring in solid cancers and vascular malformations might associate, at least in part, to ion-transport alteration.

Epidermal innervation morphometry by immunofluorescence and bright-field microscopy.

We investigated the agreement between simple indirect immunofluorescence (IF) and bright-field immunohistochemistry (BFI) on free-floating sections for intraepidermal nerve fiber density (IENFD) quantification. Fifty-five healthy subjects and 63 patients with probable small fiber neuropathy (SFN) underwent two adjacent skin biopsies at the distal leg processed by IF and BFI technique. Agreement between IENFD pairs obtained by each method was assessed by Bland-Altman testing.

Differential myelinated and unmyelinated sensory and autonomic skin nerve fiber involvement in patients with ophthalmic postherpetic neuralgia.

Postherpetic neuralgia (PHN) is a common and exceptionally drug-resistant neuropathic pain condition. In this cross-sectional skin biopsy study, seeking information on the responsible pathophysiological mechanisms we assessed how ophthalmic PHN affects sensory and autonomic skin innervation. We took 2-mm supraorbital punch skin biopsies from the affected and unaffected sides in 10 patients with ophthalmic PHN.

Trigeminal isolated sensory neuropathy (TISN) and FOSMN syndrome: despite a dissimilar disease course do they share common pathophysiological mechanisms?

Background: Patients presenting with bilateral trigeminal hypoesthesia may go on to have trigeminal isolated sensory neuropathy, a benign, purely trigeminal neuropathy, or facial-onset sensory motor neuronopathy (FOSMN), a malignant life-threatening condition. No diagnostic criteria can yet differentiate the two conditions at their onset. Nor is it clear whether the two diseases are distinct entities or share common pathophysiological mechanisms.

Afferent nerve ending density in the human laryngeal mucosa: potential implications on endoscopic evaluation of laryngeal sensitivity.

Laryngeal sensitivity is crucial for maintaining safe swallowing, thus avoiding silent aspiration. The sensitivity test, carried out by fiberoptic endoscopic examination of swallowing, plays an important role in the assessment of dysphagic patients. The ventricular folds appear to be more sensitive than the epiglottis during the sensitivity test.

Cutaneous silent period recordings in demyelinating and axonal polyneuropathies.

Objective: To investigate the cutaneous silent period (CSP), a spinal inhibitory reflex mainly mediated by A-delta fibres, in demyelinating and axonal polyneuropathy (PNP) and evaluate whether CSP parameters differ between patients with and without neuropathic pain.

Methods: Eighty-four patients with demyelinating PNP, 178 patients with axonal PNP and 265 controls underwent clinical examination, DN4 questionnaire, standard nerve conduction study, motor-root stimulation and CSP recordings from abductor digiti minimi. We calculated the afferent conduction time of CSP (a-CSP time) with the formula: CSP latency-root motor evoked potential latency.

Topiramate modulates habituation in migraine: evidences from nociceptive responses elicited by laser evoked potentials.

Background: Lack of habituation during repetitive stimulation is the most consistent interictal abnormality of cortical information processing observed in migraine. Preventive migraine treatments might act by stabilizing cortical excitability level and thus the habituation to external stimuli.

Methods: We examined the effects of preventive treatment with topiramate on migraineur's habituation to nociceptive stimulation.

Pain in the upper anterior-lateral part of the thigh in women affected by endometriosis: study of sensitive neuropathy.

Objective: To assess whether pain in the anterior-lateral part of the thigh in women affected by endometriosis is due to femoral nerve invasion by endometriotic implants.

Design: Case-control study.

Setting: Hospital.

Cutaneous innervation of the human face as assessed by skin biopsy.

The morphology of cutaneous sensory and autonomic innervation in human trigeminal territory is still unknown. The aim of this study is to describe facial cutaneous innervation using skin biopsy. This new tool could be useful in understanding the mechanisms underlying several facial pain conditions.

Mechanisms of pain in multiple sclerosis: a combined clinical and neurophysiological study.

In this clinical and neurophysiological study, we examined the clinical characteristics and underlying mechanisms of neuropathic pain related to multiple sclerosis. A total of 302 consecutive patients with multiple sclerosis were screened for neuropathic pain by clinical examination and the DN4 tool. In patients selected for having ongoing extremity pain or Lhermitte's phenomenon, we recorded somatosensory evoked potentials, mediated by Aβ non-nociceptive fibres, and laser evoked potentials, mediated by Aδ nociceptive fibres.

Laboratory tools for assessing neuropathic pain.

Neuropathic pain, i.e. pain arising as a direct consequence of a lesion or disease of the somatosensory system, affects about the 7 % of the general population.

Hyperalgesic activity of kisspeptin in mice.

Background: Kisspeptin is a neuropeptide known for its role in the hypothalamic regulation of the reproductive axis. Following the recent description of kisspeptin and its 7-TM receptor, GPR54, in the dorsal root ganglia and dorsal horns of the spinal cord, we examined the role of kisspeptin in the regulation of pain sensitivity in mice.

Results: Immunofluorescent staining in the mouse skin showed the presence of GPR54 receptors in PGP9.

Primary hypothalamic lymphoma in a patient with systemic lupus erythematosus: case report and review of the literature.

A 67-year-old female was admitted to our department with difficulty in speech, disorientation, memory loss and seizures. Blood laboratory tests revealed diabetes insipidus. This patient had been treated with steroids for systemic lupus erythematosus (SLE) for 30 years.