Grace Scott

Stalning af amyloid percursor protein to study axonal damage in mild head Injury

The most common definition of cerebral concussion is that of a transient loss of neurological function without macroscopic or microscopic abnormality in the brain. However, some patients have persistent symptoms and subtle neuropsychological deficits, particularly affecting memory. We have studied five patients aged 59-89 years who sustained mild concussive head injury and died of other causes (2-99 days post-injury). Immunostaining with an antibody to amyloid precursor protein, a marker of fast axonal transport, showed multifocal axonal injury in all five. All had axonal damage in the fornices, which are important in memory function.

Pontomedullary tears and other gross brainstem injuries after vehicular accidents

In a series of 988 autopsied victims of road crashes, there were 36 (3.6%) cases of gross primary brainstem injury. These fell into three groups. The first comprised eight cases of pontomedullary tearing without other gross brain injury: in seven of these, there were associated atlanto-occipital dislocations and/or high cervical fracture-dislocations. The usual cause appeared to be facial impact inducing acute hyperextension. Second, there were 17 cases of pontomedullary tearing associated with other brainstem lacerations and/or major damage elsewhere in the brain: in all, there were fractures of the skull base, typically transverse middle fossa fractures. Most of these injuries appeared to be due to facial impacts transmitting force to the anterior skull base, although hyperextension was also a factor in some. There was a third heterogeneous group of 11 cases with brainstem lacerations in sites other than the pontomedullary junction: in some of these it appeared that the impacts had caused skull base fractures by inducing calvarial torsion. In this series, the proportion of motorcyclists (41.7%) was double the expected figure. The use of a helmet modifies the mechanisms of impact head injury; the overall benefits of helmet use are well established, but there is need for more research on helmet design.

Consensus neuropathological diagnosis of common dementia syndromes: testing and standardising the use of multiple diagnostic criteria

Abstract. The aim of this study was to assess the variation between neuropathologists in the diagnosis of common dementia syndromes when multiple published protocols are applied. Fourteen out of 18 Australian neuropathologists participated in diagnosing 20 cases (16 cases of dementia, 4 age-matched controls) using consensus diagnostic methods. Diagnostic criteria, clinical synopses and slides from multiple brain regions were sent to participants who were asked for case diagnoses. Diagnostic sensitivity, specificity, predictive value, accuracy and variability were determined using percentage agreement and kappa statistics. Using CERAD criteria, there was a high inter-rater agreement for cases with probable and definite Alzheimers disease but low agreement for cases with possible Alzheimers disease. Braak staging and the application of criteria for dementia with Lewy bodies also resulted in high inter-rater agreement. There was poor agreement for the diagnosis of frontotemporal dementia and for identifying small vessel disease. Participants rarely diagnosed more than one disease in any case. To improve efficiency when applying multiple diagnostic criteria, several simplifications were proposed and tested on 5 of the original 20 cases. Inter-rater reliability for the diagnosis of Alzheimers disease and dementia with Lewy bodies significantly improved. Further development of simple and accurate methods to identify small vessel lesions and diagnose frontotemporal dementia is warranted.

Cervical and thoracic juxtafacet cysts causing neurologic deficits.

Study Design. Case reports and review of the literature. Objectives. To review the clinical features, treatment, and outcome of juxtafacet cysts. Summary of Background Data. There have previously been 4 reported cases of thoracic juxtafacet cysts and 19 cases of cervical juxtafacet cysts. Cervical cysts have usually originated from the cruciate ligament and caused myelopathy. Thoracic cysts are usually signaled by myelopathy. Methods. The records of the Neurosurgery Department of Royal Adelaide Hospital from 1980 through 1995 were reviewed for cases of intraspinal juxtafacet cysts. Results. Eight cases of intraspinal juxtafacet cysts were identified; six were in the lumbar spine. One patient had a cervical cyst related to a facet joint and had unilateral radiculopathy. A second patient with a thoracic cyst had the gradual onset of myelopathy. Both patients had surgical excision of the cyst without resection of the adherent dura. The symptoms and neurologic signs improved in each case. Conclusions. Cervical and thoracic juxtafacet cysts are rare lesions that are usually signaled by myelopathy. Results of surgery are excellent in most cases, even if the cyst is not completely excised.

Pathological evidence of encephalomyelitis in the stiff man syndrome with anti-GAD antibodies

We report the case of a 57 year old woman with typical clinical features of the stiff man syndrome (SMS) and antibodies to glutamic acid decarboxylase (antiGADAb), who developed a supranuclear gaze palsy, ileus and died of bronchopneumonia eight years after the onset of illness. Post mortem examination revealed perivascular lymphocyte cuffing throughout the cerebral hemispheres, brainstem and spinal cord and neuronal loss in medial anterior horns of the cervical spinal cord. These findings support the notion that the SMS and progressive encephalomyelitis with rigidity (PER) form a clinical and pathologic continuum.

The ‘rosette‐forming glioneuronal tumor’ of the fourth ventricle

Tumors containing both neuronal and glial components are a rare heterogeneous group with unique features that require further subclassification. The rosette‐forming glioneuronal tumor of the fourth ventricle is one of a number of recently described glioneuronal tumors, which has been accorded official WHO nosologic status only in 2007. We describe the clinical and pathologic features of two patients with rare rosette‐forming glioneuronal tumors of the fourth ventricle, one of which was associated with dysgenetic tricho‐rhinopharyngeal type I syndrome.

Topography and Severity of Axonal Injury in Human Spinal Cord Trauma Using Amyloid Precursor Protein as a Marker of Axonal Injury

STUDY DESIGN Axonal injury was examined in 18 human cases of acute spinal cord compression using amyloid precursor protein as a marker of AI. OBJECTIVES To topographically map and semiquantitate axonal injury in spinal cord compression of sufficient severity to produce para- or quadriplegia. SUMMARY OF BACKGROUND DATA Amyloid precursor protein is carried along the axon by fast axoplasmic transport and has been extensively used as a marker of traumatic axonal injury. METHODS The study group comprised 18 cases of spinal cord compression (17 due to fracture dislocation of the vertebral column and 1 iatrogenic compression from Harrington rods) and two normal control. All the cords were examined according to a standard protocol, and at least 10 segmental levels were immunostained using a monoclonal antibody to amyloid precursor protein and immunopositive AI was semiquantitated using a grading system to provide the axonal injury severity score (AISS). The focal injury at the site of cord compression (haemorrhage, haemorrhagic necrosis, ischaemic necrosis) was also semiquantitated to provide the focal injury area score (FIAS). AI occurring around the site of focal compression (focal axonal injury severity score or FAISS) was distinguished from AI distant to the focal injury (nonfocal axonal injury severity score or NFAISS). RESULTS All 18 cases showed widespread amyloid precursor protein immunoreactive axonal injury and the AISS ranged from 28 to 60%. In all cases, the FAISS was greater than the NFAISS and there was a statistically significant relationship between the AISS and the FIAS. CONCLUSION Acute spinal cord compression of sufficient severity to produce permanent paralysis causes widespread axonal damage that is maximal at the site of compression but also present throughout the length of the cord in segments far distant from the site of the focal injury.

Correlation of postmortem MRI and CT appearances with neuropathology in brain trauma: a comparison of two methods

Postmortem magnetic resonance (MR) scans were performed on the brains of 12 victims of fatal head injuries. These were compared with neuropathological studies of the entire brain. The first six subjects were imaged with the brain in situ and comparison was also made with antemortem computed tomography (CT). The brains from the subsequent six subjects were removed at autopsy, fixed in formalin and then imaged in a mitre box designed to overcome the problems encountered in the pilot study. Although both CT and MR imaging (MRI) detected all clinically relevant haemorrhagic lesions, many pathologically significant lesions were missed. MRI detected many more lesions than CT, but still failed to visualize areas of non-haemorrhagic axonal injury.

Septo-optic dysplasia and unexpected adult death--an autopsy approach.

A 20-year-old woman who suffered from septo-optic dysplasia died unexpectedly following a presumed viral gastroenteritis. Autopsy and neuropathological examination confirmed optic nerve hypoplasia with absence of the septum pellucidum. Marked adrenal gland hypoplasia reflected hypothalamic hypopituitarism. The small and large intestines were dilated and filled with liquid fecal material. This case demonstrates that individuals with septo-optic dysplasia may be at risk of unexpected death at all ages. The complexity of mechanisms of death in rare dysmorphic conditions may be overlooked if relevant clinical information is not available at the time of autopsy and unless specific steps are taken to clearly delineate the underlying features of the condition.

Ganglioglioma of the lateral ventricle presenting with blepharospasm : case report and review of the literature

Gangliogliomas of the lateral ventricle are unusual tumours of the central nervous system. We report a 25-year-old female who presented with a 4-week history of generalized headaches associated with left-sided blepharospasm. Clinical examination revealed bilateral papilloedema but no focal neurological deficits. Computed tomography and magnetic resonance imaging demonstrated a large lesion within the left lateral ventricle. Gross total surgical resection of the lesion was performed through a para-sagittal, trans-callosal approach. Histopathological analysis revealed the diagnosis of ganglioglioma. The post-operative course was uneventful, with complete resolution of both the headaches and blepharospasm. The presentation with an ipsilateral blepharospasm has never previously been reported in the setting of a lateral ventricular tumour.