Cough syncope and hyperventilation-induced convulsion in Chiari 1.5 malformation.

Chiari malformation type I (CM1) is defined as cerebellar tonsillar herniation below the level of the foramen magnum. Syncope, especially cough syncope, is a rare but important symptom of CM1 patients. Here, we report a CM1 patient, in combination with brainstem herniation (CM1.5), presenting with repetitive syncope who was successfully treated by decompressive surgery. A 43-year-old right-handed male, with 5-year history of repeated episodes of loss of consciousness in association with cough, was investigated. Neurological examination revealed slight muscle weakness, clumsiness, and sensory disturbance in the left upper limb. There was no sign of orthostatic hypotension or orthostatic intolerance. Cranial and spinal magnetic resonance imaging revealed a herniation of the cerebellar tonsils and a syringomyelia. Forced hyperventilation during electroencephalogram (EEG) induced brief generalized symmetric clonic convulsions with preserved consciousness, but no overt EEG seizure patterns or slow activities were found. Based on the diagnosis of CM1.5 with recurrent episodes of loss of consciousness, he underwent foramen magnum decompression. He has no recurrence of the episode after the surgery on 1 year follow-up. Decompressive surgery was an effective procedure for cough syncope and other symptoms of the current patient with CM1.5. Dissociation of cerebrospinal fluid pressure between the cranial and spinal compartments which leads further herniation of the cerebellar tonsils and subsequent compression on the cerebellum and the brainstem is considered to be the major mechanism of his cough syncope. Analysis of EEG can be useful not only to diagnose epileptic seizures but also to elucidate mechanisms of syncope and concurrent involuntary movements.

Deep slow nasal respiration with tight lip closure for immediate attenuation of severe tics.

BACKGROUND: Severe intractable tics, which are associated with Tourette syndrome and chronic tic disorder (TS/CTD), severely affect the quality of life. Common less-invasive treatments are often unable to attenuate tics with deep brain stimulation currently being the only effective treatment. We aimed to assess the anti-tic effect of deep slow nasal respiration with tight lip closure using patients with TS/CTD. METHODS: We retrospectively analyzed 10 consecutive patients (9 men, 1 woman; 23-41 years old). We instructed the patients to perform the procedure for 120 s and to obtain a video recording of before and during the procedure. The videos were used to count tics and determine lip competency or incompetency. The counted tics were rated using the modified Rush Video Rating Scale. RESULTS: Compared with before the procedure, there were significantly lower frequencies of motor and phonic tics, as well as video scored, during the procedure. Eight patients presented with lip incompetency before the procedure and none after the procedure (P = 0.041). There were no side effects associated with the procedure. CONCLUSION: Our findings indicate that deep slow nasal respiration with tight lip closure ameliorates tics in patients with TS/CTD. In accordance with our results, lip opening and oral breathing could be causes of tics, in addition to heritability. Therefore, this novel procedure could improve tics. Furthermore, our findings could contribute toward the development of tic treatments and elucidate their pathophysiology regarding the reward system, hypersensitivity, autonomic nerves, and nasal airway.

Intradural extramedullary ganglioneuroma associated with multiple hamartoma syndrome.

A 51-year-old woman presented with a rare completely intradural and extramedullary spinal ganglioneuroma associated with multiple hamartoma syndrome and manifesting as complaints of neck pain and dizziness persisting for 8 months. Magnetic resonance imaging of the spinal cord revealed an intradural extramedullary lesion at the C1 level. She underwent right suboccipital craniectomy and C1-2 hemilaminectomy to remove the tumor. Histological examination confirmed ganglioneuroma. She also suffered from multiple facial trichilemmomas, thyroid goiter, multiple polyposis of the gastrointestinal tract, and pulmonary hamartoma indicating multiple hamartoma syndrome. These benign neoplasms were treated conservatively.

Dural repair for intraspinal extradural meningeal cyst.

A 52-year-old woman presented with an intraspinal extradural meningeal cyst in the thoracolumbar region manifesting as progressive sensory disturbance of the bilateral lower extremities. Magnetic resonance imaging and computed tomography myelography showed an extradural meningeal cyst extending from the T-12 to L-4 levels in the thoracolumbar region with a dural defect and a valve-like mechanism developed in the enlarging cyst. Operative findings revealed a dural defect that allowed communication between the extradural cyst cavity and the subarachnoid space. Surgical resection of the cyst wall and repair of the dural defect resolved the symptoms.

[Three cases of de novo aneurysms].

We report 3 cases of de novo aneurysms that developed long after neck clipping of the initial aneurysms (range, 7 to 20 years, mean 12 years). Case 1 was a 58-year-old female who had undergone clipping of a ruptured aneurysm 17 years previously. Ten years later, she suffered another subarachnoid hemorrhage due to rupture of a new aneurysm, for which neck clipping was performed. Another seven years later, she had a third subarachnoid hemorrhage, and angiography revealed a new aneurysm, for which neck clipping was performed. Case 2 and 3 were both 68-year-old females who had suffered subarachnoid hemorrhage with two aneurysms and had undergone neck clipping for them respectively, 20 years and 12 years previously. Each patient was admitted to our hospital with complaints of headache, diplopia, vertigo, etc., and newly formed aneurysms were detected by magnetic resonance angiography. Conventional angiography revealed three and one new aneurysms, respectively. Since case 1 was a special case of multiple aneurysms in which lesions appeared in series rather than in parallel, all three patients harbored multiple aneurysms. It is recommended that patients with multiple aneurysms, especially those after a long period postoperatively, undergo periodic examination on an outpatient basis to detect formation of de novo aneurysms by magnetic resonance angiography or 3D-CT angiography.

Expression of c-Jun N-terminal kinases after axotomy in the dorsal motor nucleus of the vagus nerve and the hypoglossal nucleus.

c-Jun N-terminal kinases (JNKs)/stress-activated protein kinases (SAPKs) are a subgroup of mitogen-activated protein kinases (MAPKs), and important mediators of signal transduction from the cell surface to the nucleus. JNK phosphorylates the transcription factor c-Jun. c-Jun is one of the earliest and most consistent markers for neurons that respond to nerve-fiber transection. To elucidate the c-Jun metabolism after axotomy, we investigated the expression of JNKs mRNA and of JNKs and c-Jun proteins following vagus and hypoglossal nerve transection. We found that JNK 1, JNK 2 and JNK 3 mRNA were positive in the cytoplasm of neuronal and glial cells. JNK 1 and JNK 2 protein were distributed mainly in the cytoplasm of neurons and glial cells, while only JNK 3 immunoreactivity was observed intensely in the nuclei of neuronal cells. Activated JNK was also observed intensely in the nuclei of neuronal cells. In sham-operated animals, the cytoplasm of a few glial cells showed moderate immunoreactivity for activated JNK, while after axotomy the cytoplasm of all perineuronal microglial cells were stained intensely. Up-regulated c-Jun and phosphorylated c-Jun immunoreactivities were found in the nuclei of neuronal cells in the severed side of both the dorsal motor and hypoglossal nuclei. These results indicate that the principal activity of JNKs in neurons is contributed largely by JNK 3 under both normal and axotomized conditions, and that JNKs play an important role in signal transduction of perineuronal microglial cells after axotomy.

Changes in expression of p38 mitogen-activated protein kinase in the dorsal motor nucleus of the vagus nerve and hypoglossal nucleus after axotomy in adult rats.

Mitogen-activated protein (MAP) kinase cascades are activated in response to various extracellular stimuli. P38 MAP kinase is one of the MAP kinase family and is activated by proinflammatory cytokines and environmental stresses. Activating transcription factor-2 (ATF-2) is one of the targets for p38 MAP kinase. To obtain information on the role of the p38 MAP kinase in the neurons and glial cells after axotomy, we investigated changes of expression of p38 MAP kinase, MAP kinase kinase (MKK) 3, MKK4, MKK6 and ATF-2 in the dorsal motor nucleus of the vagus nerve and the hypoglossal nucleus following axotomy in rats using in situ hybridization and immunohistochemical techniques. Expression of p38 MAP kinase mRNA was observed in the neurons in control rats and showed no remarkable changes after axotomy in both nuclei. On the other hand, expression of p38 MAP kinase mRNA was observed in the perineuronal microglias after axotomy. The expression of p38 MAP kinase, activated p38 MAP kinase, MKK3 and ATF-2 were immunohistochemically observed in neurons of control rats in both nuclei. After axotomy, the expression of p38 MAP kinase, active and inactive, and ATF-2 in neurons were reduced in both nuclei, while expression of mRNA of p38 MAP kinase showed no reduction in neurons. These findings indicate that p38 MAP kinase is functionally regulated not by synthesis but by phosphorylation and regulates the activation of ATF-2 in neurons, and this cascade plays some role in retrograde neuronal reactions. Moreover, perineuronal microglial cells showed strong expression of p38 MAP kinase, active and inactive, after axotomy in both nuclei. These findings suggest that p38 MAP kinase is related to microglial cell reactions after axotomy.