Intracranial Pressure Fluctuations Mimicking Seizures in a Patient With Shunt Malfunction.

SUMMARY: This case describes a patient with new onset of multiple daily paroxysmal stereotyped episodes with alteration of arousal and responsiveness. These episodes pose a diagnostic challenge because they may be misdiagnosed as epileptic seizures because of stereotypy, similarities in semiology, risk factors such as structural abnormality, and rhythmic EEG pattern. A 20-year-old woman with Chiari malformation, spina bifida, and ventriculoperitoneal shunt presented with paroxysmal episodes of change in responsiveness, concerning for seizure activity. Anti-seizure medication was started without amelioration. During the stereotyped episodes, she suffered from paroxysmal headache, drowsiness, poor responsiveness, and relative bradycardia. EEG confirmed relative bradycardia and revealed rapid buildup of generalized rhythmic delta activity without ictal features. Improvement with ICP lowering, and lack of epileptiform activity on EEG or localizing ictal semiology, prompted obtaining shunt imaging and brain MRI. The patient was subsequently diagnosed with shunt malfunction and underwent shunt revision, resulting in complete resolution of her paroxysmal spells. Although common differential diagnosis of brief paroxysmal stereotyped spells includes seizures or psychogenic nonepileptic attacks, this patient's case demonstrates clinical paroxysms caused by intracranial pressure fluctuations (plateau waves). When evaluating patients with possible intracranial pressure abnormality for paroxysmal spells, shunt malfunction should be considered as well.

Connectivity of the human insula: A cortico-cortical evoked potential (CCEP) study.

OBJECTIVE: The human insula is increasingly being implicated as a multimodal functional network hub involved in a large variety of complex functions. Due to its inconspicuous location and highly vascular anatomy, it has historically been difficult to study. Cortico-cortical evoked potentials (CCEPs), utilize low frequency stimulation to map cerebral networks. They were used to study connections of the human insula. METHODS: CCEP data was acquired from each sub-region of the dominant and non-dominant insula in 30 patients who underwent stereo-EEG. Connectivity strength to the various cortical regions was obtained via a measure of root mean square (RMS), calculated from each gyrus of the insula and ranked into weighted means. RESULTS: The results of all cumulative CCEP responses for each individual gyrus were represented by circro plots. Forty-nine individual CCEP pairs were stimulated across all the gyri from the right and left insula. In brief, the left insula contributed more greatly to language areas. Sensory function, pain, saliency processing and vestibular function were more heavily implicated from the right insula. Connections to the primary auditory cortex arose from both insula regions. Both posterior insula regions showed significant contralateral connectivity. Ipsilateral mesial temporal connections were seen from both insula regions. In visual function, we further report the novel finding of a direct connection between the right posterior insula and left visual cortex. SIGNIFICANCE: The insula is a major multi-modal network hub with the cerebral cortex having major roles in language, sensation, auditory, visual, limbic and vestibular functions as well as saliency processing. In temporal lobe epilepsy surgery failure, the insula may be implicated as an extra temporal cause, due to the strong mesial temporal connectivity findings.

Terminal innervation of the male genitalia, cutaneous sensory receptors of the male foreskin.

PURPOSE: Understanding the types of sensory nerve termini within the glabrous skin of the human male foreskin could throw light on surgical outcomes and therapeutic possibilities for the future. Various receptor types sense changes in temperature, position, pressure, pain, light touch, itch, burning and pleasurable sexual sensations. Similarities and differences in innervation characteristics and density might become apparent when the glans penis is compared with homologous structures in the female genitalia. The aim of this study is to document the presence and characteristics of cutaneous sensory receptors in the human penile foreskin using a histopathological study of the nerve termini to achieve a more complete understanding of sensory experiences. METHODS: Foreskin samples were obtained from ten boys (aged 1-9 years) who had undergone circumcision. Informed consent was obtained from the parent/legal guardian. The samples were examined after modified Bielschowsky silver impregnation of neural tissue, and immunocytochemistry against gene protein product (PGP) 9.5 and neuron-specific enolase (NSE). RESULTS: PGP 9.5 appeared to be the most sensitive neural marker. Free nerve endings were identified in the papillary dermis visualized as thin fibers, mostly varicose, with either branched or single processes, either straight or bent. Two types of sensory corpuscle were identified: capsulated and non-capsulated. Meissner-like corpuscles were located in the papillary dermis. Capsulated corpuscles resembled typical Pacinian corpuscles, comprising a single central axon surrounded by non-neural periaxonic cells and lamellae. The capsulated corpuscles were strongly positive for PGP 9.5 and NSE. CONCLUSIONS: Free nerve endings, Meissner's corpuscles and Pacinian corpuscles are present in the human male foreskin and exhibit characteristic staining patterns.

Terminal innervation of female genitalia, cutaneous sensory receptors of the epithelium of the labia minora.

INTRODUCTION: Little information is available regarding the sensory nerve endings within the glabrous skin of the external female genitalia. The diversity of possible sensations suggests a variety of receptor types. Comprehensive knowledge of the sensory stimuli, including stimulus position, changes in temperature, pressure and pain, is critical for addressing pain and sexual function disorders clinically. The aim of this neuro-histological study is document the presence and characteristics of cutaneous sensory receptors in female genital tissue. MATERIALS AND METHODS: Labial skin samples were obtained from ten normal girls (aged 1-9 years). The specimens were waste tissue obtained during surgical intervention. They were all obtained by the senior investigator, a pediatric urologist, after the parent or legal guardian had given informed consent. The specimens were stained by Cajal-type silver impregnation and by immunocytochemistry against protein gene product (PGP) 9.5 and neuron-specific enolase (NSE). RESULTS: PGP 9.5 was the most sensitive neural marker for identifying cutaneous sensory receptors. Free nerve endings (FNEs) in the papillary dermis appeared as thin fibers, varicose, branched or single processed, straight or bent. In the labia minora, FNEs were identified in the strata basale, spinosum and granulosum of the epidermis. Non-capsulated (Meissner-like) corpuscles in the dermal papillae interdigitated with epidermal ridges of the skin. Capsulated corpuscles protruded from the deep dermis into the epidermis. Encapsulated corpuscles and cells located in the inner and outer cores were strongly positive for PGP 9.5. CONCLUSIONS: FNEs, Meissner's corpuscles and Pacinian corpuscles are present in the female labia minora and exhibit characteristic staining patterns.

Immunocytochemical characterization of pacinian-like corpuscles in the labia minora of prepubertal girls.

STUDY OBJECTIVE: To better understand the precise role of sensory corpuscles within the female external genitalia. DESIGN: After IRB approval, waste tissue samples were obtained from 10 normal girls (aged 2-9 years) who underwent surgery for labial fusion. Immunocytochemistry against protein gene product 9.5 (PGP 9.5), neuron-specific enolase (NSE), vasoactive intestinal peptide (VIP), 5-hydroxytryptamine transporter (5HTT), 5-hydroxytryptamine receptor 1A (5HT1A), Neuronal Peptide Y (NPY), neuronal nitric oxide synthase (nNOS), and estrogen receptors (ER) α and ß was performed. RESULTS: Pacinian-like corpuscles were identified in epithelium of labia minora of prepubertal girls. A central structure composed of an axon surrounded by a central core, outer core, external capsule, surrounded by encapsulated stroma, and a subsidiary innervation in the outer aspect of the corpuscle stroma stained for PGP 9.5 in the outer core and layers of the external capsule, NSE positive cells in layers of the outer core, 5HTT in stroma of the corpuscle and cells located in layers of the outer core, 5HT1A in cells of outer core, NPY in stroma of the corpuscle, and nNOS in external core and external capsule of the central structure. ERα was present in stroma, external core, and external capsule, and ERß in stroma of the corpuscle with subsidiary innervation in the stroma positive to PGP 9.5, VIP, and NPY. CONCLUSION: PGP 9.5, NSE, ERα, nNOS, and 5HTT immunoreaction detected in the outer core and external capsule could indicate these areas may play an important role in the functional aspects of the Pacinian-like corpuscle.

Innervation of the labia minora of prepubertal girls.

INTRODUCTION: Surgical and histologic sources of information give little reference to innervation, vascular, and epithelial details of the labia minora. Little is known about areas of nerve density, epithelial qualities, and vascular compartments of the labial minora that contribute to sexual arousal and orgasm. Surgical procedure development and counsel about surgical risks related to labioplasty and surgical flaps created from labial tissue may be based on inadequate information. METHODS: Labial samples from 10 normal girls (aged 2-9 years) who underwent surgery for labial fusion utilized waste tissue strips for immunohistochemical identification of S-100 and neuronal nitric oxide synthase (nNOS) in the labia minora. RESULTS: Vascular and lymphatic plexus lie within the reticular dermis, which contains a dense mesh of nerve fibers with a higher concentration of nerve fiber at the level of the subepithelial plexus. Dense innervations are located at the epidermis, extending along the basal and spinous layers of the epithelium of labia minora. Nerve bundles in the papillary dermis are associated with sebaceous and eccrine glands and nerve terminals located throughout the epithelium. The introital epithelium of the labia minora is highly innervated with widespread and intense staining, detected in the introital border of the labia minora versus the external one. The dermis appeared to display S-100 and nNOS immunolabelling. S-100 was also immunopositive in the epidermis. CONCLUSION: Labia minora is highly innervated along its entire edge. Related vascular compartment tissue involved in engorgement during sexual arousal makes this tissue important for sexual response. Labioplasty risks removal of tissue with an important contribution to sensory sexual arousal. Movement of labial tissue during genitoplasty may have different sensory outcomes dependent on which labial surface is used.

Success of treatment modalities for labial fusion: a retrospective evaluation of topical and surgical treatments.

STUDY OBJECTIVE: Standard treatment for girls with labial fusion has included topical estrogen cream, manual separation, or surgery. Side effects may limit the use of topical estrogen. Betamethasone has recently shown efficacy at separating labial fusion. Local irritation and inflammation may be an initiator of labial fusion. No adverse effects of betamethasone treatment have been documented. Long-term side effects are unknown. This study compares therapies for conservative management of labial fusion for efficacy and focuses on the response rate, time to separation, recurrence, and side effects of treatment. DESIGN: A retrospective chart review. PARTICIPANTS: One hundred fifty-one prepubertal girls, mean age 3 years (range 0.25-8.75 years) diagnosed with labial fusion. MAIN OUTCOME MEASURES: To investigate the incidence of related symptoms, length of topical estrogen or betamethasone treatment, side effects, rate of successful separation, rates of recurrence, percentage requiring surgery, and postoperative outcomes in patients with labial adhesion who underwent treatment. RESULTS: Of 151 patients with labial adhesion, 11 (7.3%) presented with urinary frequency, 30 (19.9%) with urinary tract infections, 13 (8.6%) with vaginitis, and 19 (12.6%) with post-void dripping. When compared to patients treated with betamethasone (1.3 months), patients treated primarily with premarin took nearly twice as long (2.2 months) for resolution of their adhesions. Rates of recurrence were lower for patients receiving betamethasone therapy. Side effects for estrogen therapy included breast budding and vaginal bleeding, and for betamethasone, local irritation was reported. Some patients went on to surgery and experienced recurrence after surgery. CONCLUSION: Initial comparison of topical estrogen and betamethasone treatment of labial fusion suggests that betamethasone may separate fusion quicker with less recurrence and fewer side effects than topical estrogen therapy.