We report a case and review the recent literature describing 36 patients with both Lhermitte-Duclos disease (LDD) and Cowden disease (CD). Lhermitte-Duclos disease, or dysplastic gangliocytoma, is a benign hamartomatous condition involving the cerebellum. The presenting symptoms are usually headaches, gait ataxia, and symptoms of lower cranial nerve involvement. Cowden disease is a rare autosomal dominant disease that usually presents with multiple mucocutaneous lesions. Patients with CD are prone to multiple systemic malignancies, the most common of which is breast cancer. Recent studies have demonstrated an association between LDD and CD.

A 44-year-old woman with a previous history of breast cancer, multiple benign skin lesions, Hashimoto's thyroiditis, and chronic headaches presented with exacerbation of her headaches during the previous year. Magnetic resonance imaging of the brain revealed a right cerebellar nonenhancing mass and an acquired tonsillar herniation.

The patient underwent resection of the right cerebellar mass, posterior fossa decompression, C1 and C2 laminectomies, and a duraplasty. Pathologic examination confirmed LDD. The patient recovered well after surgery, with immediate improvement of her headaches.

The association between LDD and CD has been under-recognized and under-reported. Recognition of this association has direct clinical relevance, because diligent monitoring of individuals with LDD and CD may lead to the early detection of systemic malignancies.

We propose a simple modification to an established blood-brain barrier disruption (BBBD) animal model that allows us to use ketamine/xylazine as the anaesthetic agent, therefore decreasing the complexity and the cost of the model, while maintaining similar efficiency.

Sixty-two Long Evans rats were anaesthetized by intraperitoneal injection of ketamine/ xylazine. Osmotic BBBD was performed by administering 25% mannitol into the internal carotid artery in a retrograde fashion from the external carotid. The infusion rate of mannitol, as well as the duration was adjusted in a stepwise fashion to identify optimal parameters for BBBD and minimize complications. As a supplementary step to previously reported models, a vascular clip was applied to the common carotid artery prior to the infusion of mannitol, therefore isolating our model system from the depressant hemodynamic effects of ketamine/xylazine. Evans blue dye was used to control for BBBD intensity.

Using this model at an initial infusion rate of 0.15 ml/sec, a significant incidence of brain hemorrhage (75%) and a death rate of 62.5% were observed. Decreasing the infusion rate in a stepwise fashion, 0.08 ml/sec was found to produce optimal BBBD, as demonstrated by Evans blue staining. At this rate, 6/7 animals depicted grade II staining, whereas one animal depicted grade IV.

The application of a clip to the common carotid artery prior to mannitol infusion allowed us to isolate the cerebral circulation from the depressant hemodynamic effects of ketamine/xylazine. This supplementary step produced consistent and efficient BBBD in our animal model.

The lateralizing value of the motor automatisms is generally doubted in most patients with temporal lobe epilepsy. However, subgroup analysis of the seizures of temporal lobe origin suggests a role for motor automatisms in discriminating seizures of neocortical versus mesial temporal lobe origin.

Video-EEG of a patient with well-defined neocortical temporal lobe epilepsy was reviewed to assess the localizing value of motor automatisms.

We report a patient with left upper extremity motor automatisms and clonic movements of the proximal left lower extremity with altered awareness as the sole manifestations of right temporal neocortical seizures.

Early onset unilateral motor automatisms without dystonic posturing can localize the seizure origin to the contralateral temporal lobe neocortex.

Lymphocytic hypophysitis is an autoimmune condition that commonly presents in women of childbearing age as hypopituitarism and a sellar mass.

A 66-year-old woman presented with anterior pituitary dysfunction. Computed tomography imaging revealed a small hypodensity that was not felt to be the cause of the pituitary dysfunction. Eight years later, her vision rapidly deteriorated and MRI showed a pituitary mass lesion causing optic chiasm compression. Histological examination of the partially resected gland revealed evidence of lymphocytic hypophysitis.

Our patient is an example of the variable presentation and course of lymphocytic hypophysitis. Such a long latent period between the initial presentation of adenohypophysial hypofunction and optic chiasm compression due to an enlarging pituitary mass has not been reported.

Hemangiopericytoma is an aggressive vascular tumour that rarely affects the central nervous system and is even more rarely spinal in presentation. The clinical features of a patient with a recurrent extraspinal hemangiopericytoma presenting with an epidural spinal cord compression by local invasion are described, including a review of the literature on metastatic hemangiopericytoma to the spine.

A case of a 53-year-old male, with a recurrent extraspinal hemangiopericytoma which metastasized to the thoracic spine five years after detection of the primary tumour is presented. A chart review was conducted where all pertinent history, physical, laboratory, and radiological data were collected. A Pub-Med search using the keyword “hemangiopericytoma” identified all reported cases documenting clinical features, treatment, recurrence and outcome with respect to metastatic hemangiopericytoma to the spine.

Nine patients have been reported to have metastatic hemangiopericytoma to the spine. The median patient age was 47 years and there was a slight male preference. An unusual feature of the hemangiopericytoma is the prolonged period, up to 16 years, between the diagnosis of the primary hemangiopericytoma and the metastases to the spine. All patients were treated with a combination of radiation and surgery.

Hemangiopericytomas show a slow clinical evolution with a strong propensity to relapse long after previous treatment and thus, once identified, prolonged follow-up for recurrence is indicated. A close follow-up of these patients is required because of frequent recurrences and delayed metastases even if the primary lesion was well-controlled. Although overall uncommon, hemangiopericytoma should be kept in mind in the differential diagnosis of vascular epidural spinal cord tumours.

To report an unusual case of large synchronous lumbar disc herniation in adult twins.

Case report and relevant literature review.

The authors report a case of adult female monozygotic twins who synchronously presented with huge lumbar disc herniation. Rather than being a curious coincidence, this case highlights the potential importance of hereditary factors in the causation of lumbar disc degeneration. The relevant literature regarding the role of genetic factors and their relationship to other risk factors in lumbar disc degeneration is reviewed and discussed.

Synchronous lumbar disc herniation in adult twins is a rare event. It, however, sheds light on the role of genetic factors in disc degeneration.

Endozepine-4 stupor is a rare disorder. It presents with recurrent episodes of stupor and coma. Endozepine-4 levels are elevated during an attack with electroencephalogram showing fast activity during the ictal phase. There is no specific treatment and various drugs have been tried without long-term benefit.

We used the vigilance promoting drug modafinil to prevent such attacks in a case presented with endozepine stupor.

The vigilance promoting drug modafinil prevented recurrent episodes of stupor and coma in this patient at a dose of 200 mg/day for a period of six months. Upon withdrawal of the drug, the patient experienced recurrent episodes and on reinstitution of modafinil she has remained asymptomatic in the following one year.

Neurenteric cysts are congenital cysts of the central nervous system that are believed to be of endodermal origin. In this report we present the unique case of a supratentorial neurenteric cyst that is contained entirely within the brain parenchyma.

Apatient presented with an intraparenchymal cystic lesion that was subsequently identified as a neurenteric cyst. This lesion is reviewed in light of the available literature.

A 35-year-old female presented with a one year history of progressive headaches and seizure-like episodes. Her examination revealed no deficits. Magnetic resonance imaging showed a 4 cm x 4 cm x 4 cm cystic lesion within the parenchyma of the right frontal lobe. A right frontal craniotomy and complete excision of the cystic lesion was performed. Pathologic examination confirmed that it was a neurenteric cyst. Postoperatively the patient's symptoms improved.

Review of the literature revealed this to be the first case of a surgically excised, pathologically confirmed supratentorial neurenteric cyst, contained entirely within the brain parenchyma.

Double cortex syndrome is a malformation in which there is a band of subcortical heterotopic grey matter separated from the cortex by white matter. The functional activity of the heterotopic neurons is unclear.

A 13-year-old female was evaluated for seizures. The EEG showed bifrontal spike wave disturbance. Band heterotopia, in association with mild reduction of sulcation of the cerebral hemispheres, was found on MRI. Psychological assessment indicated the presence of variable cognitive abilities, with verbal IQ [82] generally better than nonverbal IQ [59], and specific difficulties in language comprehension and mathematics.

Functional MRI was used to localize the areas of language and motor activation. The language activation paradigm was a visual verb generation task with a visual fixation baseline. The motor paradigm consisted of alternating blocks of sequential finger tapping and rest. Coronal functional and anatomical images were obtained.

The motor paradigm produced activation of the primary motor cortex, the band heterotopia and the supplementary motor cortex. The language paradigm produced activation of the left inferior frontal gyrus and left supplementary motor area, but not of the band heterotopia.

The activation of heterotopic grey matter during a motor task demonstrates a hemodynamic association with motor activity and suggests that this tissue may be functional. Such association was not seen with the language task. We speculate that later maturing functions such as language are restricted in their development to the normal situated superficial cortex in our patient.

Central neurocytoma is a tumour that typically occurs in young adults in close association with the lateral and third ventricles of the cerebrum.

We report the unusual case of a central neurocytoma that developed in the fourth ventricle of a 59-year-old woman and metastasized to the upper cervical canal. Subtotal excision and adjuvant radiotherapy were used to treat the lesion. Microscopic evaluation, discussion of the pathologic differential diagnosis and theories of the histogenesis of the tumour are presented. Results and

Fourth ventricular neurocytoma is rare and has only been reported twice previously. It appears most likely that this tumour arises from subependymal progenitor cell lines.

Spontaneous intracranial hypotension (SIH) is a well-recognized neurologic disorder that typically presents with orthostatic headaches, low cerebral spinal fluid pressures and distinct abnormalities on magnetic resonance imaging.

We present a case of a rare presentation of SIH.

A 49-year-old man presented with a two week history of orthostatic headaches that rapidly progressed to encephalopathy and coma, requiring intubation. Neuroimaging revealed abnormalities typical of SIH; diffusely enhancing pachymeninges, subdural fluid collections, and descent of the brain. Treatment with an epidural blood patch reversed his coma within minutes. Following a second blood patch, the patient became asymptomatic. No cerebral spinal leak could be identified on magnetic resonance imaging or on a nuclear medicine technetium cerebral spinal fluid flow study. At six month follow-up, he remained symptom free.

The mechanism of coma in SIH is presumed to be compression of the diencephalon from downward displacement of the brain. Although it is very unusual for patients with SIH to present with coma, it is important to recognize since the coma may be reversible with epidural blood patches.

Devic's syndrome is a demyelinating disease of the spinal cord and optic nerves. It tends to have a poor prognosis, probably due to the occurrence of necrosis within lesions. There is no proven effective treatment although relapses are commonly treated with corticosteroids and people with recurrent attacks may be managed with chronic immune suppressing treatments. Intravenous gamma globulin (IVIG) and plasma exchange are reasonable treatment options because Devic's syndrome is believed to be antibody mediated. We report two patients of Devic's syndrome that stabilized following initiation of monthly IVIG.

A 42-year-old woman with a 23 year history of Devic's syndrome continued to have frequent attacks of optic neuritis unresponsive to daily corticosteroids and azathioprine. Since initiation of monthly IVIG 5/2 years ago she has had no further definite attacks. She has also noted minimal improvement in color perception.

A 58-year-old woman with a three year history of Devic's syndrome experienced five attacks during the first 16 months of disease. Monthly IVIG was associated with complete cessation of relapses and significantly improved neurological status over one year of treatment.

Because active Devic's disease often results in severe, permanent neurological impairment, preventive intervention should be considered. These cases suggest that IVIG may be effective in preventing attacks and possibly in enhancing neurological recovery. Randomized controlled trials will be needed to confirm this and to determine optimal dosing and treatment duration.