Hereditary neuropathy with liability to pressure palsies in a Turkish patient (HNPP): a rare cause of entrapment neuropathies in young adults.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant nerve disease usually caused by 1,5 Mb deletion on chromosome 17p11.2.2-p12, the region where the PMP-22 gene is located. The patients with HNPP usually have relapsing and remitting entrapment neuropathies due to compression. We present a 14-year-old male who had acute onset, right-sided ulnar nerve entrapment at the elbow. He had electrophysiological findings of bilateral ulnar nerve entrapments (more severe at the right side) at the elbow and bilateral median nerve entrapment at the wrist. Genetic tests of the patient demonstrated deletions in the 17p11.2 region. The patient underwent decompressive surgery for ulnar nerve entrapment at the elbow and completely recovered two months after the event. Although HNPP is extremely rare, it should be taken into consideration in young adults with entrapment neuropathies.

An upper thoracic spinal cord tumor presenting as hemifacial hyperhidrosis.

BACKGROUND: Hyperhidrosis as the sole presenting symptom of an upper thoracic intramedullary tumor has never been reported in the English literature. CASE DESCRIPTION: A 17-year-old boy presented with a long history of hemifacial flushing and hyperhidrosis on the left side of his face and neck. The MRI revealed a large spinal cord tumor at the T1-T2 levels. The patient underwent total excision of the intramedullary tumor via a posterior myelotomy. The histopathological diagnosis was low-grade astrocytoma. The symptoms resolved immediately after the surgery and did not return during the follow-up period of 9 months. CONCLUSIONS: We suggest that sympathetic irritation on the left side is the mechanism behind this clinical presentation. Its unusual presentation and lack of motor and sensory deficits resulted in delayed diagnosis of this potentially disabling lesion. When autonomic dysfunction of the face and neck is encountered, in addition to the cranial and cervical regions, the upper thoracic levels should be investigated using MRI.

Posterior fossa dermoid cysts causing cerebellar abscesses.

Dermoid cysts are uncommon tumors, and posterior fossa dermoid cysts may rarely cause abscess formation or formation of daughter abscesses within the cerebellum. At present, there are only 16 cases with posterior fossa dermoid cysts causing cerebellar abscesses reported in the literature. Two cases, 22 and 14 months old, with posterior fossa dermoid cysts and dermal sinus causing multiple cerebellar abscesses are reported. In the first one, there was also marked hydrocephalus. Retrospective examination of the patients revealed pinpoint-sized dimples in their suboccipital regions. Both patients were treated with antibiotics and underwent posterior fossa surgery. In the patient with marked hydrocephalus, ventriculoperitoneal shunting was performed after treatment of the infection. Both patients were neurologically normal, and there were no complaints, except a light learning difficulty in the patient with ventriculoperitoneal shunting, 133 and 34 months after surgery, respectively. Early detection of congenital dermal abnormalities along the craniospinal axis by routine examination of newborns is highly important before development of serious complications. Because surgery is the only effective treatment modality for these lesions, radical excision should be performed in all cases to avoid tumor recurrence. However, subtotal excision may be performed in selected cases, because the cyst capsule may adhere firmly to vital structures.

Giant subdural empyema in a child: a case report.

An unusual case of a giant (8 x 6 x 6 cm) frontoparietal SDE of Streptococcus pneumoniae in a 17-month-old child is reported. The initial diagnosis was made with emergency CT. The purulent material was removed via a frontoparietal craniotomy. A series of postoperative MR imaging showed the gradual reduction in size of the lesion, although collapsed capsule, fibrous thickening of meningeal structures and associated displacement of the underlying brain persisted. The child was symptom-free in a follow-up period of 15 months. This case showed that SDE may reach a giant size and thus may mimic an intra-axial lesion; the coronal MR imaging is a more reliable diagnostic tool than the emergency axial CT in giant SDE of upper convexity localization, and the clinical improvement may be more impressive than the radiological changes.

Contrast-enhanced MR 3D angiography in the assessment of brain AVMs.

BACKGROUND AND PURPOSE: Digital subtraction angiography (DSA) is the current reference standard for the diagnosis, assessment, and management of brain arteriovenous malformations (AVMs). The purpose of this study was to compare the diagnostic utility of three-dimensional (3D) time-of-flight (TOF) magnetic resonance angiography (MRA) and contrast-enhanced 3D MRA in patients with intracranial arteriovenous malformations (AVMs) in different sizes and locations. The AVM diagnosis was proved via DSA and almost half of the patients had also hematoma. MATERIALS AND METHODS: Two radiologists, experienced on neurovascular imaging and independent from each other, retrospectively reviewed two MRA techniques and DSA with regard to the assessment of feeding arteries, AVM nidus, and venous drainage patterns on 20 patients with 23 examinations by scoring system. Disagreements were resolved by consensus. RESULTS: An excellent agreement between contrast-enhanced MRA and DSA was found in order to assess the numbers of arterial feeders and draining veins (Spearman r=0.913, P<0.001). The average scores in contrast-enhanced MRA for feeders, nidi, and drainers were respectively 2.26, 2.69, and 2.48, while in TOF-MRA they are 1.96, 1.35, and 0.89, respectively. CONCLUSION: Compared to TOF-MRA, 3D contrast-enhanced MRA is useful for visualization by subtraction technique of malformation components presented by hematoma or by haem product. On the other hand, for the cases presented by slow or complex flow that is especially in around or nidi or around the venous portion is also advantageous because of the independence from flow-related enhancement. Therapeutic effects were clearly demonstrated in three follow-up patients. A major limitation of this technique is the low spatial resolution. Since there is such a limitation, arterial feeder of a case with micro-AVM is not detected by contrast-enhanced MRA and nidus for the same case was observed retrospectively. In this respect, we believe that 3D contrast-enhanced MRA is a less invasive and inexpensive angiographic tool, but not a safe substitute for DSA. Yet, it can be a beneficial supplement to DSA in patients with cerebral AVMs at both initial diagnosis and at follow-up processes after therapy.

Microneurosurgical training model in fresh cadaveric cow brain: a laboratory study simulating the approach to the circle of Willis.

BACKGROUND: Residents of neurosurgery need many years to develop microneurosurgical skills, and laboratory training models are essential for developing and refining surgical skills before clinical application of microneurosurgery. A simple simulation model is needed for young residents to learn how to handle microneurosurgical instruments, and to perform safe dissection of intracranial vessels and nerves. METHODS: The material consists of a 2-year-old fresh cadaveric cow cranium. A 4-step approach was designed to dissect the internal carotid artery and its proximal branches, the optic nerve, the optic chiasm, and the pituitary stalk. RESULTS: The model simulates standard microneurosurgery using a variety of approaches to vessels and neural structures in and around the circle of Willis of the human brain. CONCLUSION: The cadaveric cow brain, besides being cost-effective, represents a fairly useful method to accustom residents of neurosurgery, especially junior residents, to dissecting intracranial vessels and nerves, and it simulates intracranial microneurosurgical procedures performed in the human brain.

Nocardial brain abscess: review of clinical management.

Nocardiosis has become a significant opportunistic infection over the last two decades as the number of immunocompromised individuals has grown worldwide. We present two patients with nocardial brain abscess. The first patient was a 39-year-old woman with systemic lupus erythematosus. A left temporoparietal abscess was detected and aspirated through a burr-hole. Nocardia farcinica infection was diagnosed. The patient had an accompanying pulmonary infection and was thus treated with imipenem and amikacine for 3 weeks. She received oral minocycline for 1 year. The second patient was a 43-year-old man who was being treated with corticosteroids for glomerulonephritis. He was diagnosed with a ring-enhancing multiloculated abscess in the left cerebellar hemisphere, with an additional two small supratentorial lesions and triventricular hydrocephalus. Gross total excision of the cerebellar abscess was performed via a left suboccipital craniectomy. Culture revealed Nocardia asteroides, and the patient was successfully treated with intravenous ceftriaxone, then oral trimethoprime-sulfamethoxazole for 1 year. The clinical course, radiological findings, and management of nocardial brain abscess are discussed in light of the relevant literature, and current clinical management is reviewed through examination of the cases presented here.

A laboratory training model for interhemispheric-transcallosal approach to the lateral ventricle.

Laboratory training models are essential for developing and refining surgical skills before clinical application of microneurosurgery. Our aim is to train residents of neurosurgery to be familiar with a basic microneurosurgical technique in access to the lateral ventricle via a transcallosal approach. The training material consists of a 2-year-old fresh cadaveric cow cranium. A four-step approach was designed to simulate microneurosurgical dissection along the falx to visualize cingulated gyri, callosomarginal and pericallosal arteries in order to perform callosotomy and access to the lateral ventricle, and finally to the foramen of Monroe. We conclude that the model perfectly simulates standard microneurosurgical steps in interhemispheric-transcallosal approach to the lateral ventricle and to the area of the foramen of Monroe.

Paraplegia due to spinal subdural hematoma as a complication of posterior fossa surgery: Case report and review of the literature.

Although blood contamination of cerebrospinal fluid (CSF) after an intracranial operation is possible, development of a symptomatic spinal hematoma after a posterior fossa surgery has never been reported. A 43-year-old woman underwent a posterior fossa tumor removal in the prone position with no intraoperative difficulty. On the second postoperative day, she complained of severe epigastric pain and developed a rapid onset of paraplegia with anesthesia below the thoracic 5 spinal level. The emergency cranial and spinal MRIs revealed a spinal extramedullary hemorrhage spreading to the whole spinal regions, just sparing the cauda equina area. There was a prominent localized hematoma formation surrounding and compressing the spinal cord at the upper thoracic levels, which was evacuated via an urgent laminectomy. The patient showed partial neurological recovery after the decompression. Development of the spinal hematoma was explained by the movement of blood from the tumor bed into the spinal canal under the effect of gravity, during or after the operation. A 30 degrees head elevation might facilitate the accumulation of blood. Localization of the hematoma formation may be caused by the fact that the upper thoracic levels constitute the apex of the kyphosis. We conclusively suggest that a spinal hematoma should be taken into consideration as a rare but potentially severe complication of a posterior fossa surgery. Meticulous hemostasis and isolation of the surgical area from the spinal spaces are essential. Overdrainage of CSF should be abandoned. Postoperatively, patients should be monitored for spinal findings as well as cranial signs.

Large intradiploic growing skull fracture of the posterior fossa.

Growing skull fractures (GSFs) are rare complications of head injury and mostly occur in infancy and early childhood. Location in the posterior fossa and intradiploic development of a GSF is very uncommon. We report a 7-year-old boy with a large, 9 x 7 x 4-cm, occipital intradiploic GSF. The lesion developed progressively over a period of 5 years following a documented occipital linear fracture. This case of a GSF developing from a known occipital linear fracture demonstrates that a GSF may reach a considerable size and, although uncommon, intradiploic development and occipital localization of a GSF is possible.

[Pediatric head injuries: a retrospective analysis of 280 patients.]. / Çocukluk çagi kafa travmalari: 280 olgunun retrospektif degerlendirmesi.

BACKGROUND: To assess etiological factors, clinical features, radiological findings and recovery rates in pediatric head injuries. METHODS: Patients (n =280) with head injuries (age range: 0 - 16 years) hospitalized in Trakya University Department of Neurosurgery between January 1995 and 2004 were analyzed statistically. RESULTS: According to Glasgow Coma Scale (GCS) the patients had minor (GCS: 13- 15 ; 70.1% ), moderate (GCS: 9- 12; 17,1% ), or severe (GCS: 3 to 8; 6,8% ). head injuries The most common etiological factor was fall from a height (34,3%); and the most frequently associated injury was extra-spinal skeletal injury (12,9%). Fifty-one patients (18,2%) underwent neurosurgical operation. 87.5% of them recovered completely, while 12,5% showed partial recovery or died, as graded by Glasgow Outcome Scale (GOS). There was a moderately strong correlation between initial GCS and GOS (r=0,53, p=0,01). CONCLUSIONS: Nearly half of the pediatric head injuries were caused by falls with good prognoses. In the school age, motor vehicle accident (MVA) was the most frequent trauma type. MVA was the most serious type of trauma as demonstrated by its low GCS and GOS scores. Polytraumas, subdural hematomas, cerebral contusions, subarachnoid or intracerebral hemorrhages, cerebral edemas, diffuse axonal injuries, and any cranial lesion which required surgery were found to be related with poor prognosis.

Contralateral subdural effusion after aneurysm surgery and decompressive craniectomy: case report and review of the literature.

We report a complication of decompressive craniectomy in the treatment of aneurismal subarachnoid hemorrhage (SAH) and accompanying middle cerebral artery (MCA) infarction. A 56-year-old man presented with subarachnoid hemorrhage and right sylvian hematoma. He was diagnosed with high-grade SAH and medical therapy was employed. He showed rapid clinical deterioration on day 9 of his admission. Computed tomographic scans showed right MCA infarction and prominent midline shift. Because of the patient's rapidly worsening condition, further evaluation to find origin of SAH could not be obtained, and decompressive right hemicraniectomy was performed. During sylvian dissection, right middle cerebral and posterior communicant artery aneurysms were detected and clipped. One week after operation, a contralateral frontoparietal subdural effusion and left to right midline shift was detected and drained through a burr-hole. Through successive percutaneous aspirations, effusion recurred and complete resolution was achieved after cranioplasty and subduroperitoneal shunt procedures. Decompressive craniectomy is generally accepted as a technically simple operation with a low incidence of complications. In the light of this current case, we hypothesize that a large craniectomy may facilitate the accumulation of recurrent effusion on contralateral side creating a resistance gradient between two hemispheres. This point may be especially true for subarachnoid hemorrhage cases requiring aneurysm surgery. We conclusively suggest that subdural effusions may be resistant to simple drainage techniques if a large contralateral craniectomy does exist, and early cranioplasty may be required for treatment in addition to drainage procedures.