Symptomatic sacral Tarlov cyst: Case report and review of literature

Tarlov cysts are a common finding in MRI. Most of them are asymptomatic but in some cases can cause pain in urogenital region. Diagnosis and treatment are controversial and most of the symptomatic cases are not well diagnosed and treated because of unawareness of neurosurgeons about them. Treatment of symptomatic TC is effective and good results have been published with percutaneous and surgical techniques. A case of a young woman with a symptomatic sacral cyst treated surgically successfully is presented and literature about it is reviewed.     

Sacral Tarlov cysts: Neurophysiology abnormalities and correlation with pelvic sensory and visceral symptoms

Background and purpose

Recent studies suggest a possible association between Tarlov cysts (TCs), usually considered as incidental radiological findings, and neurological symptoms such as pain, numbness and urogenital complaints. The aim was to explore the relationship between TCs and sacral nerve root functions using pelvic neurophysiology tests, and to correlate changes with clinical symptoms and magnetic resonance imaging (MRI) findings.

Methods

Consecutive patients with sacral TCs, referred for pelvic neurophysiology testing and presenting with at least one symptom related to the pelvic area, participated in a cross-sectional review of symptoms using validated questionnaires. Findings of pelvic neurophysiology (pudendal sensory evoked potentials, sacral dermatomal sensory evoked potentials, external anal sphincter electromyography) and urodynamics testing were collected retrospectively. The relationship between neurophysiology, MRI findings and patients' symptoms was assessed using Fisher and ANOVA tests.

Results

Sixty-five females were included (mean age 51.2 ± 12.1 years). The commonest symptom was pain (92%). Urinary (91%), bowel (71%) and sexual (80%) symptoms were also frequently reported. Thirty-seven patients (57%) had abnormal neurophysiology findings reflecting sacral root dysfunction. No association was seen between MRI findings (size, location of the cysts, severity of compression) and neurophysiology. A negative association was observed between neurophysiology abnormalities and occurrence of urgency urinary incontinence (p = 0.03), detrusor overactivity (p < 0.01) and stress urinary incontinence (p = 0.04); however, there was no association with voiding difficulties.

Conclusions

Contrary to current understanding, TCs are associated with injury to the sacral somatic innervation in the majority of patients with presumed symptomatic cysts. However, urinary incontinence is unlikely to be related to TC-induced nerve damage.     

Infraspinatus muscle palsy involving suprascapular nerve,brachial plexus or cervical roots related to inflammatory or mechanical causes: Experience of 114 cases

ObjectivesTo report a large series of neurogenic infraspinatus muscle (ISM) palsy, in order to improve knowledge of diagnosis, diverse etiologies and care management.MethodsClinical and electrodiagnostic (EDX) data for 114 cases of ISM palsy were collected over a 21-year period. Cases were attributed to 4 clinical conditions: (1) isolated suprascapular nerve mononeuropathy (n = 48), (2) multiple mononeuropathies (n = 33), (3) plexus lesions (n = 17), and (4) cervical radiculopathy (n = 16). These were related to 2 mechanisms: inflammatory (dysimmune) and mechanical.ResultsGroup 1 cases were younger, had the most severe ISM palsies, were mostly related to inflammatory lesions (81%) such as neuralgic amyotrophy (NA), and frequently had delayed diagnosis because disability was mild. Group 2 cases were all related to inflammatory lesions and had slightly less severe ISM palsies that were frequently hidden by winged scapula. In groups 3 and 4, ISM palsies were milder and all cases were related to mechanical lesions such as brachial plexus trauma or C4-C5-C6 radiculopathy. In these cases, deltoid and ISM palsies were equal in frequency and severity whereas biceps brachii impairment was less frequent and much milder. Deltoid palsy frequently appeared predominant as compared with ISM palsy, because upper limb elevation palsy was more disabling than external rotation palsy.ConclusionsISM palsy is a rare condition, often under-diagnosed and misidentified. The 4 main conditions of ISM palsy may be recognized by careful clinical, EDX and other examinations when necessary. Analysis of the present series highlights some clinical and EDX points that should help non-specialist and even specialist clinicians who are faced with this rare condition, to distinguish mechanical and inflammatory causes, and thus adapt patient management accordingly.     

Simultaneous MR neurography and apparent T2 mapping of cervical nerve roots before microendoscopic surgery to treat patient with radiculopathy due to cervical disc herniation: Preliminary results

There is no imaging modality to quantitatively evaluate compressed cervical nerve roots in cervical radiculopathy. Here we sought to evaluate the usefulness of simultaneous apparent T2 mapping and neurography with nerve-sheath signal increased with inked rest-tissue rapid acquisition of relaxation-enhancement imaging (SHINKEI-Quant) to evaluate compressed nerves quantitatively in patients with cervical radiculopathy due to cervical disc hernia before microendoscopic surgery.One patient with cervical radiculopathy due to cervical disc hernia before microendoscopic surgery and 5 healthy subjects underwent simultaneous apparent T2 mapping and neurography with SHINKEI-Quant. The patient was a 49-year-old man with severe right upper arm pain and numbness. Based on MRI images, we suspected right C7 radiculopathy due to C6-7 cervical disc hernia. The T2 relaxation times of the cervical dorsal root ganglia of the brachial plexus bilaterally at C5–C8 were measured.We observed no significant differences in T2 relaxation times between the nerve roots on the left and right at each spinal level with values in healthy subjects. In our patient, neurography revealed swelling of the right C7 nerve, and a prolonged T2 relaxation time compared with that of the contralateral, unaffected C7 nerve. We performed microendoscopic surgery and the symptoms improved.We were able to evaluate the injured nerve root quantitatively in a patient with cervical radiculopathy using the SHINKEI-Quant technique, being the first study to our knowledge to show the usefulness of this technique to evaluate cervical radiculopathy quantitatively before microendoscopic surgery.     

Vagus nerve stimulation in children with refractory epilepsy: unusual complications and relationship to sleep-disordered breathing

Background Vagus nerve stimulation (VNS) is approved for use in patients with refractory epilepsy over the age of 12 years. While this procedure is widely used, there is little data on adverse events in young children. Materials and methods A retrospective chart review was conducted on 26 children who had VNS implantation for refractory epilepsy from 1998 to 2004. Results Ages ranged from 3 to 17 years (16 boys and 10 girls). Seventy-seven percent had moderate to severe mental retardation. Sixty-five percent had more than 30 seizures per month. Symptomatic-generalized epilepsy was the predominant epilepsy syndrome seen in 77% of children. The duration of VNS treatment ranged from 1 month to 8 years (mean = 3.5 years). Twenty of 26 patients (77%) were on rapid-cycling mode. More than 50% reduction in seizure frequency was noted in 54% with two patients achieving seizure freedom. Twenty-three percent had less than 50% seizure reduction. Four patients were able to terminate seizures with use of the magnet. VNS was removed from one patient because of intractable cough persisting in spite of stimulation being turned off for 1 month. Another patient had it removed twice for infection. Obstructive sleep apnea (OSA) was observed in four patients (15%) after placement of VNS. Conclusion VNS appears to be an effective treatment for children with refractory epilepsy. Development of intractable cough in one patient in spite of device being turned off and recurrent infection-related removal in another are unusual complications. Polysomnography before implantation of VNS should be considered to identify patients with pre-existing OSA. This work was presented as an abstract at the American Epilepsy Society Meetings in Washington, DC, 2–6 December 2005. S. Neff, deceased during publication of this article.     

Spontaneous intracranial hypotension due to sacral diverticula: Two-case history and a pocket-sized review

Spontaneous intracranial hypotension is an increasingly recognized cause of atypical, secondary headaches. Nevertheless, its clinical and imaging spectrum is far from an exhaustive definition, ranging from straightforward cases with unambiguous findings and prompt response to treatment to more challenging ones, requiring advanced, more complex imaging and targeted therapies. We describe two unusual cases as a cue to draw a literature-based, practical approach to the management of the syndrome.     

Trauma-induced auditory nerve degeneration due to cerebellopontine（CP） angle manipulations： clinical and experimental experience

In order to investigate the pathophysiologic mechanisms responsible for trauma-induced hearing disturbances due to auditory nerve degeneration, we established for the first time a rat experimental model in which auditory nerve degeneration due to compression injury of the cerebellopontine (CP) angle portion of the auditory nerve can be quantitatively evaluated. In this paper, I demonstrate our clinical experience in CP angle surgery and some of the results of our experiments performed on this rat experimental model. Trauma-induced hearing loss in CP angle operations has long been regarded as a sort of unavoidable "natural course" and therefore hopeless. I believe that this pessimistic view should be challenged and changed through new approaches in scientific research.     

The spectrum of Charcot-Marie-Tooth disease due to myelin protein zero: An electrodiagnostic,nerve ultrasound and histological study

Objective

Nerve ultrasound (US) data on myelin protein zero (MPZ)-related Charcot-Marie-Tooth disease (CMT) are lacking. To offer a comprehensive perspective on MPZ-related CMTs, we combined nerve US with clinics, electrodiagnosis and histopathology.

Brachial plexopathy due to malignant peripheral nerve sheath tumor in neurofibromatosis type 1: case report and subject review

Neurofibromatosis type 1 (NF1) is a common tumor predisposition syndrome affecting approximately 1 in 4,000 persons. It is an autosomal-dominant disorder with half of the cases resulting from spontaneous mutations. This genetic defect leads to the formation of benign tumors or neurofibromas of the peripheral nervous system. Dermal neurofibromas may cause local discomfort and itching but are rarely associated with neurological deficit and do not undergo malignant change. The more extensive plexiform neurofibromas produce neurological complications in 27%-43% of patients with NF1 and may undergo malignant degeneration in 5% of cases. Patients with NF1 who develop pain or new neurological symptoms should have a rapid and thorough assessment for malignancy. In this report, we illustrate this point by presenting a patient who developed acute shoulder pain and weakness due to malignant degeneration of a plexiform neurofibroma involving the left brachial plexus, and review the literature on this subject.     

Development of the spinal nerves of the larval lamprey: IV. Spinal nerve roots of 21-mm larval and adult lampreys, with special reference to the relation of meninges with the root sheath and the perineurium

Spinal nerve roots of 21-mm larval and adult lampreys were electron microscopically studied. In 21-mm larval lampreys, each ventral and dorsal rootlet contains axons of various diameters enclosed together as groups in individual troughs of a Schwann cell cytoplasm, lying in direct contact with one another, and is further ensheathed entirely by a basal lamina. Dorsal roots possess visceral axons, while ventral roots lack them. In adult lampreys the ventral and dorsal roots possess individual sheaths for larger somatic axons, each being surrounded by a single Schwann cell and the basal lamina and separated from one another by a considerable amount of connective tissue. Visceral fibers are present in both the dorsal and ventral roots of adult lampreys. They aggregate to form fascicles that lie among somatic axons, being separated from them. Two layers of the meningeal tissue invaginate to form a root sheath around the distal portion of individual dorsal and ventral roots of 21-mm larval lampreys. In adult lampreys the sheath is similarly formed but extends over most of the dorsal and ventral roots. The perineurium is not developed in 21-mm larval lampreys, but is present and ensheaths only the proximal portion of spinal nerve trunks outside the meninges in adult lampreys: it is completely absent along most of the length of peripheral nerves. In both larval and adult lampreys, the outer cell layer of the root sheath is open-ended near the middle of nerve roots with respect to the extramedullary connective tissue space. Similar loosening of the cellular barrier is seen along blood vessels. Thus, the outer meningeal fibrous layer is directly continuous with the extra medullary connective tissue space by way of the inner fibrous layer of the root sheath.     

Transition from child and adolescent mental health care to adult services for young people with Attention-Deficit/Hyperactivity Disorder (ADHD) or Autism Spectrum Disorder (ASD) in Europe: Barriers and recommendations

Transition in mental health care is the process ensuring continuity of care of a young patient arriving at the CAMHS (Child and Adolescent Mental Health Service) age boundary within mental health services. Transition refers to a transfer to an adult mental health service (AMHS), to private care or other mental health community services. A transition plan can also lead to a managed end of specialized care with involvement of a general practitioner or social services. For young people with a diagnosis of ADHD (Attention Deficit Hyperactivity Disorder) or ASD (Autism Spectrum Disorder), two disorders that persist into adulthood, an optimal transition would ensure continuity of care or facilitate access to specialized care in the case of a discharge. Transition typically occurs during adolescence, a known sensitive period when young people may experience major changes at several levels: physiological, psychological and social. Any barrier in the transition process resulting in discontinuity of care may worsen the symptoms of ADHD or ASD and can ultimately adversely affect the global mental health of young people with such neurodevelopmental disorders. The objectives of this narrative review are: 1/to identify the barriers in the transition process in mental health services often faced by young people with these two disorders; 2/to highlight specific recommendations for strengthening the CAMHS-AMHS interface that have been proposed by various countries in Europe.