Michel Reznik

Clinical and radiological aspects of dysplastic gangliocytoma (Lhermitte-Duclos disease): a report of two cases with review of the literature.

Two cases of Lhermitte-Duclos disease confirmed by biopsy are reported. Review of the 58 published cases shows that the disease can manifest itself only by signs of increased intracranial pressure. Cerebellar symptoms are not constant. Computed tomographic (CT) scans suggest the diagnosis by showing a posterior fossa lesion, iso- and hypodense, partially calcified, and not enhanced by contrast medium. Magnetic resonance imaging (MRI) seems to define limits of the lesion better than CT scanning and could improve the surgical approach. Surgical excision of the lesion is the only satisfactory treatment. The postoperative prognosis is usually favorable.

Mri-Pathological Correlations in Acute Traumatic Central Cord Syndrome: Case Report

SummaryAnte- and post-mortem MRI and detailed pathological examination were performed in a patient with a typical acute traumatic central cord syndrome (ATCCS) after a minor hyperextension injury to the neck who died 60 h later from heart failure. T2-weighted MRI showed a central hyperintense area at C3–4. There were disc protrusions, but no vertebral fracture or displacement. Histopathology disclosed severe axonal swelling and oedema in the dorsolateral fasciculi and, to a lesser degree, in the dorsal columns. In addition, an area of recent necrosis was found in the right anterior horn at C4–5. These findings suggest that the pathological hallmark of typical ATCCS is mechanical axonal disruption at a segmental level, but that more severe trauma may be accompaniedby tissue destruction.

Idiopathic hypertrophic cranial pachymeningitis mimicking multiple meningiomas: case report and review of the literature.

Abstract A case of idiopathic hypertrophic cranial pachymeningitis with an unusual and misleading manifestation is reported. Computed tomography scan, angiographic and magnetic resonance imaging findings were suggestive of multiple meningeal neoplasms and a correct diagnosis was made only after meningeal biopsy. This 44-year-old patient had a previous history of an ill-defined systemic disorder associating episcleritis, erythroderma nodosa and multiple peripheral arthritis. We review previous reports of idiopathic cranial pachymeningitis with emphasis on radiological investigation techniques, histopathology and possible dysimmune mechanisms of pathogenesis.

Myositis during Borrelia burgdorferi infection (Lyme disease).

During the second stage of an illness caused by Borrelia burgdorferi, a young woman developed a myopathic syndrome characterised by severe muscular pains, incapacitating weakness of the proximal limb and the neck, as well as the bulbar muscles and elevated serum CK levels. Muscle biopsy revealed a non-inflammatory necrotising myopathy. B. burgdorferi infection was confirmed by a considerable rise of specific IgG antibodies. A course of high dose steroids alleviated the myalgias, but paresis began to improve only after treatment with antibiotics. Our observations confirm that B burgdorferi can cause, through an undertermined mechanism, a necrotising myopathy, in addition to the wide spectrum of already known neurological complications.

Clinical and neuropathological parameters affecting the diagnostic yield of nerve biopsy

The value of nerve biopsy in the investigation of peripheral neuropathies is an important and controversial issue, partially obscured by the large variations in the diagnostic yield routinely reported for this procedure. The aim of this study was to evaluate the clinical and neuropathological parameters affecting the yield of nerve biopsy. We compared the experience of two independent neuropathology laboratories with different patient recruitment and neuropathological methods over 11 years (01/1987-12/1997). Clinicopathological correlations were studied retrospectively in 355 patients. Using the same criteria of evaluation, contributive biopsies accounted for 35.5% in one laboratory, and 47.3% in the other. Clinical parameters affecting the yield of nerve biopsy were: (a) the presumptive diagnosis at time of referral for biopsy; (b) the distribution of symptoms; and (c) the interval between disease onset and biopsy. Greater yield was associated with clinically suspected vasculitis, inflammatory demyelinating neuropathy or hereditary sensorimotor neuropathies. Contributive findings were more often reported with multifocal or asymmetrical presentations, and onset-to-biopsy interval of less than 6 months. The contribution of nerve biopsy varied according to neuropathological techniques: (a) serial sections on frozen. paraffin-embedded and resin-embedded material improved sensitivity for interstitial pathology: (b) combined muscle biopsy increased sensitivity in the detection of vasculitis; and (c) teasing of nerve fibers added critical information to other classical techniques in only 4/102 cases.

Shunt-related abdominal metastasis of cerebral teratocarcinoma: report of an unusual case and review of the literature.

OBJECTIVE AND IMPORTANCE Internal drainage of cerebrospinal fluid to the abdominal cavity via a ventriculoperitoneal shunt (VPS) is a common procedure for therapy of obstructive hydrocephalus; because this condition is often caused by brain tumors blocking the natural cerebrospinal fluid pathways, the VPS as an artificial anastomosis can provide the means for the spreading of tumor cells by the cerebrospinal fluid. We report the case of a VPS-related abdominal metastasis of a teratocarcinoma and review the pertaining literature. CLINICAL PRESENTATION AND INTERVENTION A 24-year-old man with a history of three brain tumors that were operated on when the patient was 14, 21, and 23 years of age developed an acute ileus 7 months after VPS insertion for cerebral teratocarcinoma. Intraoperatively, a massive abdominal tumor was observed, which turned out to be a peritoneal metastasis of the aforesaid brain tumor. The patient died as a result of his illness 1 month later. RESULTS To date, 58 VPS-related metastases of brain tumors have been described. The male-to-female ratio is 1.6:1, the mean age at shunt insertion is 12.2 years, and the interval between shunt operation and diagnosis of metastases is 16.8 months. During the observation time, 69.2% of the patients died as a result of their illness or abdominal metastases. The most common sources of the metastases were germinomas (27.7%), medulloblastomas (19.1%), and endodermal sinus tumors (10.3%). CONCLUSION The presented case is only the second VPS-related abdominal spreading of a cerebral teratocarcinoma. Metastases via VPS are rare but should be considered as a possible complication and mode of systemic spread in patients with primary intracranial malignancy.

Pathology of Primary Reticulum Cell Sarcoma of the Human Central Nervous System

Among 6000 consecutive autopsies, 9 cases of primary reticulum cell sarcoma of the central nervous system were discovered. There were 5 males and 4 females from 17 to 68 years old, and the mean clinical duration was 5 months. Gross examination revealed a wide range of lesions in the cerebral hemispheres, cerebellum and spinal cord. Histologically, the same type of malignant reticulum cell proliferation was encountered in every case.

Infantile and juvenile presentations of Alexander's disease: a report of two cases.

We describe 2 new cases of Alexanders disease, the first to be reported in Belgium. The first patient, a 4‐year‐old girl, presented with progressive megalencephaly, mental retardation, spastic tetraparesis, ataxia and epilepsy; post‐mortem examination showed widespread myelin loss with Rosenthal fibers (RFs) accumulation throughout the neuraxis. She was the third of heterozygotic twins, the 2 others having developed normally and being alive at age 5 years. The second patient developed at age 10 years and over a decade spastic paraparesis, palatal myoclonus, nystagmus, thoracic hyperkyphosis and thoraco‐lumbar scoliosis with radiological findings of bilateral anterior leukoencephalopathy. Brain stereotactic biopsy at age 16 years demonstrated numerous RFs. With these 2 cases, we review the literature on the various clinico‐pathological conditions reported as Alexanders disease. We discuss the nosology of this entity and the pathogeny of RFs formation and dysmyelination. Clues to the diagnosis of this encephalopathy in the living patient are briefly described.

Primary intracerebral osteosarcoma

A 64‐year‐old man was admitted because of a rapidly progressive left side hemiparesis. A computed tomography scan showed a right side intrathalamic mass, partially calcified. Surgical specimen proved to be an osteosarcoma. The patient died 3 days later from an acute myocardial infarct. Autopsy failed to discover any other significant lesion. A review of the literature disclosed that this case might be the second published primary intracerebral osteosarcoma and the first with immunocytochemical analysis.

Neuropathology in seven cases of locked-in syndrome

The neuropathology of 7 cases of the locked-in syndrome has been reviewed and compared with 3 cases of akinetic mutism, all of them occurring after brainstem vascular disease (9 infarctions, 1 hematoma). Including other published cases of verified locked-in syndrome, the extent of lesions has been studied in order to understand, among other symptoms, the preservation of consciousness, voluntary eye movements, auditory comprehension and vital functions. It is concluded that, at the time of autopsy, although most of the clinical signs can be explained by the pathological findings, it is not yet possible to individualize specific brainstem lesions responsible for this syndrome.