Intradural spinal arachnoid cyst contributing to sudden paraparesis.

BACKGROUND: Spinal arachnoid cysts are cystic lesions filled with cerebrospinal fluid that contributes to neurological deficits depending on their size/location within the spinal canal. Here, we report a patient with a spinal subarachnoid cyst who suddenly developed paraparesis. CASE DESCRIPTION: A 37-year-old female with a thoracic spinal arachnoid cyst at the T7 level suddenly developed lower abdominal pain followed by immediate paraparesis. Two weeks following the onset of symptoms, she underwent a T6-T8 laminectomy; this included with full cyst excision. By the 4th postoperative week, her signs/symptoms fully resolved. CONCLUSIONS: A 37-year-old female with a T7 thoracic spinal subarachnoid cyst who presented with acute paraparesis regained normal function 2 weeks following a T6-T8 laminectomy.

Usefulness of contralateral carotid angiography in revascularization therapy of acute internal carotid artery occlusion.

OBJECTIVE The vascular lumen of an acutely occluded internal carotid artery (ICA) generally contains numerous thrombi. Therefore, carotid angiography on the affected side during revascularization therapy of acute ICA occlusion has a potential risk of causing distal embolization. In this study the authors propose the use of contralateral carotid angiography. METHODS Six patients with acute ICA occlusion underwent revascularization therapy using a stent retriever or Penumbra system. Revascularization therapy was performed with placement of a 9-Fr balloon-guiding catheter (BGC) in the affected ICA and a 4-Fr diagnostic catheter in the contralateral ICA. During the procedure, the 9-Fr BGC was kept inflated, and all control angiography was performed from the 4-Fr diagnostic catheter. After thrombectomy, contralateral carotid angiography combined with manual aspiration from the 9-Fr BGC was performed to assess the presence or absence of residual thrombi in the affected ICA. The 9-Fr BGC was deflated only after the complete absence of residual thrombi in the affected ICA was confirmed. RESULTS The time required for introducing the 4-Fr diagnostic catheter into the contralateral ICA was within a few minutes in all patients. Residual thrombi in the affected ICA were found in 3 of 6 patients. The residual thrombi in these 3 patients were completely removed; thus, distal embolization was prevented. CONCLUSIONS Contralateral carotid angiography is useful for avoiding distal embolization during revascularization therapy of acute ICA occlusion. Further studies involving a larger number of patients are warranted to verify the clinical efficacy of this contralateral carotid angiography.

Regrowth and progression of multiple calcifying pseudoneoplasms of the neuraxis: Case report.

BACKGROUND: Calcifying pseudoneoplasms of the neuraxis (CAPNONs) are clinically rare, nonneoplastic, noninflammatory-calcified lesions of the central nervous system. Resection of a lesion usually indicates good prognosis without recurrence. However, we experienced a unique case of CAPNON that repeatedly recurred after resection. CASE DESCRIPTION: A 52-year-old woman with recurrence of an undiagnosed brain tumor, which was resected 12 years ago, was admitted to our institution. Three calcifying lesions along with perifocal edema were detected in the frontal interhemispheric fissure. We performed the second surgery for total removal of the lesions. The patient was diagnosed with CAPNON on the basis of the clinical features of the lesions and medical examination results. However, 14 months after the second surgery, recurrence occurred, and the patient has been followed up conservatively thereafter. CONCLUSION: CAPNONs may indicate healing process associated with an unidentified agent. Maximum resection, including an unidentified agent, is necessary to prevent recurrence.

Successful Combination of Pallidal and Thalamic Stimulation for Intractable Involuntary Movements in Patients with Neuroacanthocytosis.

OBJECTIVE: Neuroacanthocytosis (NA) is a rare neurodegenerative disease that involves severe involuntary movements including chorea, dystonia, and trunk spasms. Current treatments are not effective for these involuntary movements. Although there are a few reports on the use of deep brain stimulation to treat patients with NA, the optimal stimulation target is not yet definitive. Some authors have reported successful improvement of NA symptoms with stimulation of the globus pallidum interna, and others have reported a reduction in trunk spasm with stimulation of the ventralis oralis complex of the thalamus. We investigated whether the optimal target is well defined for NA. METHODS: We describe the effect of combination stimulation of the globus pallidum interna and the ventralis oralis complex of the thalamus in 2 patients with NA who presented with severe intractable involuntary movements. RESULTS: Gpi stimulation alone was an insufficient effect for trunk spasm and/or chorea. Vo complex stimulation given without Gpi stimulation resulted in improvement of trunk spasm after 2 weeks and might also have had an incomplete effect on involuntary movement including a chorea. The combination of Gpi and Vo complex stimulation reduced the trunk spasms and chorea. This improvement was maintained at 3 months after surgery. The Unified Huntington's Disease Rating Scale score at 1 year after surgery was lower than that before surgery. CONCLUSIONS: Gpi stimulation appears to be insufficient to control violent involuntary movements; therefore, combined GPi and Vo complex stimulation provided some moderate advantage over Gpi stimulation alone.

Endovascular microcatheter provocation test for the diagnosis of glossopharyngeal neuralgia.

OBJECTIVE: Glossopharyngeal neuralgia (GN) is a rare disease often clinically misdiagnosed as trigeminal neuralgia. An endovascular provocative test has been applied to assist in making the diagnosis of GN caused by vascular compression. We similarly used endovascular provocative techniques to identify the causative vessel and to evaluate the indication for microvascular decompression (MVD) in two patients. METHODS: Two patients had severe retroauricular and pharyngeal pain that could not be controlled by medical therapy. Magnetic resonance imaging and magnetic resonance angiography showed a high-riding right posterior inferior cerebellar artery (PICA). We decided to apply the endovascular provocation test to prove the assumed GN and identify potential indications for MVD. RESULT: A loop of the PICA appeared to compress the glossopharyngeal nerve in the first patient. In this patient, a microcatheter was inserted into the right PICA, decreasing the GN. The patient underwent MVD, resulting in complete pain relief. In the second patient, a microcatheter was inserted into the right PICA, and an attack of typical GN occurred, with pain in the posterior region of the tongue, tonsils, oropharynx, and larynx. The patient's severe pain was clearly different from this typical GN caused by the microcatheter provocation test, and MVD was not performed. CONCLUSION: The diagnosis of GN is sometimes complex. The endovascular provocative technique may allow identification of GN caused by vascular compression.

Neuromodulation using intrathecal baclofen therapy for spasticity and dystonia.

Intrathecal baclofen (ITB) therapy is a treatment for intractable spasticity due to a variety of causes. Continuous intrathecal administration of baclofen, an agonist of the inhibitory neurotransmitter γ-aminobutyric acid, inhibits excitation of motor neurons at the spinal level and thus suppresses spasticity. This therapy was introduced clinically in the Europe and the United States in the 1990s, and was finally approved by the Japanese Ministry of Health, Labour and Welfare in Japan in 2005. Clinical use has been permitted since 2006, and reports of therapeutic efficacy are now appearing in Japan. ITB therapy is a non-destructive treatment that enables administration of baclofen from an implantable pump under the control of a programmer, and represents an outstanding treatment method offering both reversibility and adjustability. Indications for ITB therapy have been expanding in recent years to include not only spasticity, but also various causes dystonia. And ITB therapy can greatly improve activities of daily living and quality of life, and this treatment is attracting attention as a neuromodulatory therapy that also affects metabolic and respiratory functions and even state of consciousness. We here report the surgical methods and therapeutic outcomes for 22 patients who underwent ITB therapy for spastic and dystonic patients in our hospital, together with an investigation of the effects on metabolic and respiratory functions.

Spinal syringomyelia following subarachnoid hemorrhage.

Subarachnoid blood has been reported as a cause of chronic spinal arachnoiditis. Although syringomyelia has been thought to be caused by spinal arachnoiditis, reports of syringomyelia following aneurysmal subarachnoid hemorrhage (SAH) are very rare. We describe two patients with syringomyelia associated with chronic spinal arachnoiditis following SAH. From January 2001 to December 2010, 198 patients with aneurysmal SAH were treated at Kinki University School of Medicine. Two of the 198 patients had syringomyelia following aneurysmal SAH; thus the rate of syringomyelia associated with aneurysmal SAH was 1.0%. Patient 1 was a 54-year-old woman who presented with back pain, back numbness and gait disturbance 20 months after SAH. Her MRI revealed syringomyelia of the spinal cord from C2 to T10. She underwent shunting of the syrinx to the subarachnoid space. Patient 2 was a 49-year-old man, who was admitted to the hospital with headache, diplopia, hoarseness, dysphagia and ataxia five months after SAH. MRI revealed syringomyelia from the medulla oblongata to C6, and an enlargement of the lateral and fourth ventricles. After foramen magnum decompression and C1 laminectomy, a fourth ventricle-subarachnoid shunt was placed by insertion of a catheter. Spinal arachnoiditis and spinal syringomyelia are rare but important chronic complications after SAH.

Hemiparesis caused by cervical spontaneous spinal epidural hematoma: a report of 3 cases.

We report three cases of spontaneous spinal epidural hematoma (SSEH) with hemiparesis. The first patient was a 73-year-old woman who presented with left hemiparesis, neck pain, and left shoulder pain. A cervical MRI scan revealed a left posterolateral epidural hematoma at the C3-C6 level. The condition of the patient improved after laminectomy and evacuation of the epidural hematoma. The second patient was a 62-year-old man who presented with right hemiparesis and neck pain. A cervical MRI scan revealed a right posterolateral dominant epidural hematoma at the C6-T1 level. The condition of the patient improved after laminectomy and evacuation of the epidural hematoma. The third patient was a 60-year-old woman who presented with left hemiparesis and neck pain. A cervical MRI scan revealed a left posterolateral epidural hematoma at the C2-C4 level. The condition of the patient improved with conservative treatment. The classical clinical presentation of SSEH is acute onset of severe irradiating back pain followed by progression to paralysis, whereas SSEH with hemiparesis is less common. Our cases suggest that acute cervical spinal epidural hematoma should be considered as a differential diagnosis in patients presenting with clinical symptoms of sudden neck pain and radicular pain with progression to hemiparesis.

[Microvascular decompression of trigeminal neuralgia caused by vascular compression which penetrated the trigeminal nerve: a case report].

A rare case of trigeminal neuralgia, caused by a branch of the superior cerebral artery running through the trigeminal nerve, was successfully treated by microvascular decompression. The nerve gap at the arterial penetration point was widened by the dissection of the epineurium to obtain sufficient mobilization of the artery and decompression of the nerve. This technique would be useful for securing neurovascular decompression, as well as for avoiding postoperative hypesthesia owing to the neurotomy described in previous reports. Preparing for such a rare case is important as preoperative magnetic resonance imaging (MRI) often fails to disclose precise diagnoses.

Cervical myelopathy caused by dropped head syndrome. Case report and review of the literature.

The authors present a rare case of cervical myelopathy caused by dropped head syndrome. This 68-year-old woman presented with her head hanging forward. After 1 month, she was admitted to the medical service because of head drop progression. Examination of biopsy specimens from her cervical paraspinal muscles showed nonspecific myopathic features without inflammation, and isolated neck extensor myopathy was diagnosed. The patient's condition did not respond to the administration of corticosteroids. During follow up as an outpatient, the patient's head drop continued to gradually progress. At 1 year after onset, she developed bilateral weakness of the upper and lower extremities, clumsiness of the hands, and gait disturbance. A radiograph of the cervical spine obtained in a standing position showed a pronounced kyphotic deformity and instability at the level of C4-5. Magnetic resonance imaging demonstrated spinal cord compression at C-3 and C-4. The patient underwent a C3-4 laminectomy and occipitocervicothoracic fixation. Gait and hand coordination gradually improved, and she was able to walk with no support 1 month postoperatively. Surgical fixation was beneficial in this patient with dropped head syndrome, myelopathy, and cervical instability.