Hereditary neuropathy with liability to pressure palsy: fulminant development with axonal loss during military training.

Hereditary neuropathy with liability to pressure palsy (HNPP) is characterised by recurrent mononeuropathies following minor trauma. We describe a case of fulminant HNPP beginning on the first day of military physical training. Protracted weakness, muscle atrophy, hand contractures, and multifocal sensory loss developed during a further three weeks of basic training. Nerve conduction changes were typical of HNPP, but without segmental slowing. Electromyographically, there was prominent acute denervation in muscles of the hands and right shoulder. Sural nerve biopsy demonstrated tomaculae and remyelination. Genetic testing revealed PMP-22 gene deletion. This case report demonstrates that HNPP can present with rapidly progressive peripheral nerve dysfunction and electrophysiological evidence of focal axonal loss.

Intrasellar vascular malformation mimicking a pituitary macroadenoma.

We report the case of a 61-year-old man with an unusual intrasellar vascular malformation, who presented with symptoms of hypopituitarism and whose neuroradiologic evaluation mimicked a pituitary macroadenoma. The histopathologic and radiologic findings are discussed. This rare lesion should be considered in the diagnosis of a sellar mass lesion.

Neurophysiological evidence of spared upper motor neurons after spinal cord injury.

Fourteen cats were subjected to a moderate (100 gm-cm; n = 7) or a severe (600 gm-cm; n = 7) spinal cord injury at the C4-C5 level using a weight drop technique. Somatosensory evoked potentials (SSEPs) were recorded after stimulation of the median nerve in the forearm. The SSEPs were measured in each animal before and after the injury. Motor evoked potentials (MEPs) were recorded from forearm extensor muscles after transcranial magnetic stimulation of the motor cortex. The SSEPs and The MEPs were measured in each animal before and after the injury under ketamine-based anesthesia. After the moderate injury (n = 7), 83% of the animals (6/7) maintained the SSEPs and 100% (7/7) maintained the MEPs. Postoperatively, only one animal who lost the SSEPs post-injury became tetraplegic. The remainder were neurologically intact. In the severely injured animals (n = 7), 5/7 of animals lost SSEPs and subsequently became tetraplegic. The MEPS were maintained in 3/5 (60%) of these tetraplegic animals. Two of seven animals (40%) in this group did not lose SSEPs or MEPs and recovered with no clinical deficit. Our data show a good correlation between the presence of SSEPs and functional recovery in the injured groups. The presence of MEPs in 3/5 (60%) of the tetraplegic animals may imply the existence of functionally active motor fibers after severe spinal trauma.

Association of systemic lupus erythematosus and gluten enteropathy.

Systemic lupus erythematosus presents various clinical manifestations. The coexistence of systemic lupus erythematosus and celiac sprue has been rarely reported. We present a patient who had systemic lupus erythematosus with malabsorption demonstrating characteristic clinical and pathologic findings of celiac sprue (gluten enteropathy).

Effect of 21-aminosteroid pretreatment in compression trauma to the spinal cord.

The present work was performed to establish whether pre-injury administration of the 21-Aminosteroid, U-74389F, is beneficial for treatment of acute spinal cord trauma in rats, as it has been demonstrated that the bolus administration of the same compound one hour after injury facilitates the return of the spinal cord function as measured by electrophysiological recordings in this compression animal model of spinal cord trauma. Cortical somatosensory evoked potentials (CSSEPs) were recorded as an indicator of spinal cord function before and after a severe compression injury. Vital signs and the CSSEPs were monitored up to five hours post-injury. U-4389F treatment was given as a single injection (15 mg kg-1) one hour prior to the injury which was followed by a continuous infusion (3 mg kg-1h-1) during the procedure. The CSSEPs were abolished immediately after this injury both, in the untreated and treated animal groups. The majority of the treated animals (80%) demonstrated recovery of the CSSEPs within the second hour post-injury. The control group showed 40% recovery at this time period. At five hours post-injury, recovery rates were 47% and 87% for control and treated groups respectively. We conclude that the administration of the 21-Aminosteroid, U74389F, one hour prior to spinal cord injury facilitates the return of spinal cord function as measured by CSSEPs in a compression rat model of acute spinal cord trauma, supporting and verifying our previous experiences using the same compound as i.v. bolus injections one, two and three hours after the trauma, respectively.

Immunostaining for Leu-7 in the diagnosis of thyroid carcinoma.

The morphologic distinction between benign and malignant thyroid lesions can be difficult. Immunostaining for the Leu-7 antigen has been reported to be useful in the diagnosis of thyroid carcinoma. To test the specificity of immunostaining for Leu-7 in the diagnosis of thyroid carcinoma, we studied formalin-fixed, paraffin-embedded tissue specimens obtained from 66 thyroid lesions using the HNK-1 monoclonal antibody to Leu-7 and an avidin-biotin-complex technique. Positive reactivity for Leu-7 was seen in 11 (33%) of 33 benign thyroid conditions and in 27 (82%) of 33 thyroid carcinomas. We conclude that immunostaining for Leu-7 is not specific in the diagnosis of thyroid carcinoma.

Pigmented villonodular synovitis presenting as a subcutaneous mass at the knee.

We describe a 45-year-old man who developed a painful mass about his knee. Magnetic resonance imaging demonstrated a well defined lesion, and biopsy confirmed a diagnosis of pigmented villonodular synovitis. Our case exemplifies a primary synovial process presenting as a subcutaneous tumor. Such diagnostic possibilities, although rare, should be more widely known.