Nicola Rizzuto

Opsoclonus and Palatal Myoclonus during Prolonged Post-Traumatic Coma

A case of opsoclonus and palatal myoclonus following blunt head injury is described. The syndrome appeared 1 month after the injury and lasted unchanged until death. Postmortem examination showed the presence of widespread lesions of the brain stem affecting the dentato-rubro-olivary system. The main clinical features of the syndrome and their anatomical correlates are discussed.

The effects of 2,5-hexanedione on axonal regeneration after nerve crush in the rat.

SummaryThe pattern of recovery of myelinated axons in the posterior tibial nerve after crushing was studied in rats chronically intoxicated with 2,5-hexanedione. It was given for 2 weeks before curshing (200 mg/kg i.p. 5 times a week) or additionally for two further weeks after the nerve crush. Two animals were examined from each group at approximately, 1, 2, 3, 4, and 8 weeks later.Return of function in poisoned animals was slower than in the controls. The numbers of regenerating myelinated fibres was severely reduced in poisoned animals, up to 4 weeks later, but by 8 weeks the numbers equalled those in the control nerves. Marked impairment of initiation of neurite outgrowth was found, but once begun, axonal growth was comparable to controls and myelination occurred normally. Above the crush for 10 mm, filament-filled axonal swellings were found in poisoned animals accompanied by varying amounts of retrograde axonal degeneration. These findings are discussed in relation to the role of normal neurofilaments in axonal growth and the effects of probably cross-linking of these by 2,5-hexanedione on regnerating neurites.

Lumbar epidural ewing sarcoma

SummaryThe clinicopathological findings in a child with extraskeletal Ewing sarcoma are described.The patient complained of pain in the lower back and difficulty walking. An extraskeletal, epidural, friable tumor, 2–3 cm long was removed from the epidural space. It had no relationship with the bone structures. Light and electron microscopic examination of the tumor led to the diagnosis of Ewing sarcoma. The morphological aspects of this neoplasia and the problem of the differential diagnosis with other small cell tumors of the epidural space are discussed.ZusammenfassungEs wird ein Ewing-Sarcom, welches sich außerhalb des Skelettes bei einem Kind entwickelte, geschildert. Klinisch manifestierte es sich als epidurale Masse. Bei der chirurgischen Exploration zeigte sich ein brüchiges Gewebe, welches 2–3 cm lang war und nicht vom Knochen ausging. Lichtmikroskopisch und elektronenmikroskopisch ließ sich die Diagnose eines Ewing-Sarcoms stellen.Es werden morphologische Aspekte und differentialdiagnostische Überlegungen in Zusammenhang mit Tumoren des Epiduralraumes diskutiert.

Friedreich’s Ataxia. A Light- and Electron Microscopic Study of Peripheral Nerve Biopsies

Sural or superficial peroneal nerve biopsies of patients with clinical diagnosis of Friedreichs ataxia were studied. Patients were divided in two groups, typical and abortive forms: loss of fibers accompanied by axonal atrophy and segmental demyelination are the basic changes in both groups, although the decrease in number of myelinated fibers was most severe in typical FA. In the cases with slower progression there is a tendency to form onion bulb complexes.

Chronic relapsing polyneuritis

SummaryThe clinico-pathologic findings in two patients with chronic relapsing polyneuritis are reported.The patients had several attacks of diffuse polyneuritis followed by partial recovery. The tendency to persisting disability increased following relapses and in the late stages there was a progressive motor deterioration suggesting a continuing activity of nerve damage.The biopsies of peripheral nerves, obtained in the advanced stages of the disease, showed severe loss of myelinated fibers and aspectes of repeated demyelination and remyelination, with formation of onion bulb complexes. Completely demyelinated axons together with remyelinating fibers as well as remyelinated fibers invested by macrophages were seen at the same time.The clinical and pathological evidences suggest that in chronic relapsing polyneuritis, at least in the late stages of the disease, besides clinical relapses, there is a continuing activity of nerve damage. Thus, due to this ongoing activity of demyelination and remyelination, the elimination of the supernumerary Schwann cells does not take place satisfactorily and hypertrophic changes of the nerve fascicles are produced.

Gliomatosis cerebri diffusa

SummaryThe clinico-pathologic findings in an additional case of gliomatosis cerebri are reported: a 60-year-old woman died 8 months after the onset of a progressive deterioration of both the neurologic and mental conditions.Neuropathologic examination disclosed wide demyelination of both hemispheres, communicating through the corpus callosum, extending downward along the internal capsule to the brainstem structures. Cellular stains showed the presence of elongated astrocytes, multinucleated cells, mitotic and anaplastic figures, involving the demyelinated areas and the neighboring regions, and allowed the diagnosis of gliomatosis cerebri diffusa.The nosologic and pathogenetic aspects of this rare entity are discussed.

Diffuse rosenthal fiber formation in adults

SummaryA 20-year-old woman was admitted to the Hospital because of tetraparesis and respiratory failure, requiring mechanical ventilation. No disturbances of consciousness were associated. She had a residual left lower limb paresis from the age of 14, lasting unchanged until this final episode of bulbar paralysis. She did not recover: death occurred 3 months later.Neuropathological examination revealed Rosenthal fibers in the gray and white matter with typical perivascular and subpial distribution. Areas of mild demyelination were found in the periventricular white matter of the centrum ovale and in the hilum of the nucleus dentatus. In the brain stem the highest density of Rosenthal fibers was found in the medulla where the deposits extended deeply into parenchyma.The relationship of this case with Alexanders disease is discussed.

Seitelberger’s Infantile Neuroaxonal Dystrophy

A case of Seitelberger’s infantile neuroaxonal dystrophy is reported. The histopathological features comprise a large amount of axonal spheroids, mainly in the gray matter of the CNS, associated with