Jean-Marie Gerard

Acute ischemic brachial plexus neuropathy following radiation therapy

Breast carcinoma is prominent among cancers where brachial plexopathy commonly occurs. Tumor invasion or postradiation fibrosis are the most common etiologies,’.* and both produce slowly progreesive neurologic symptoms and signs. Acute ischemia due to occlusion of the subclavian artery is an extremely rare cause of postradiation brachial plexopathy. Case report. In 1967, a 34-year-old woman developed a left areolar mammary carcinoma and was treated by a 4,000-rad irradiation of the breast and axillary area, followed by a radical mastectomy. Both treatments were well tolerated. Eight yeare later, a recurrence (or a second carcinoma) was found in the right breast and treated by mammectomy followed by a 4.000-rad irradiation of the breast area. This treatment was followed by a lymphedema of the right upper limb. In February 1988, &r carrying heavy objects and holding the left arm outstretched above her shoulder, she noted painlese weaknees of the left arm; decreased temperature sense of the left hand and fingers developed within a few days. Except for slight eeneory improvement in the third and fourth fingers, the manifestations remained unchanged until we first examined her in April 1988. Neurologic abnormalities, which were confined to the left upper limb, consisted of attophy and fasciculations of interosseous muscles; distal weakness grade 315 of the entire hand and the flexor of wrist and fingers; weakness grade 415 of the extenmr of wrist and proximal muscles of the arm (MRC scale); reduction of pinprick, touch, and thermic perception in the hand and touch perception in the entire forearm and aboli’ion of all tendon reflexes in the limb. There was painlese induration of the left supraclavicular fossa, and humeral, radial, and cubital pulse were absent. Blood preaeure was 140/80 on the right and 80/? on the left side. General examination findings were normal except for the lymphedema of the right upper limb. EMG showed fibrillations and resting positive sharp waves in distal muscles, and giant motor unit potentials in both distal and proximal segments of the left upper limb. No myokymic discharges were observed left paracervical muscles were normal. Sensory nerve potentials recorded at the wrist were 3.5

Central core disease: A study of clinically unaffected muscle

Abstract Bilateral pes cavus in a 48-year-old women, with no muscle weakness, was the sole manifestation of central core disease. Characteristic histological changes were found in gastrocnemius and in the clinically unaffected biceps brachii. Histochemical observations confirmed the exclusive location of central cores in type I fibres. In type II fibres there was progressive atrophy and rarefaction, either by longitudinal splitting or by conversion from type II to type I. Increased collateral branching of subterminal motor axons supports the hypothesis of the neurogenic nature of the disease. Both central cores and targets may represent stages of a non-specific metabolic disorder occurring within restricted areas of fibres having high mitochondrial enzyme activity.

Changes in motor innervation and histochemical pattern of muscle fibers in some congenital myopathies

Changes in motor innervation were compared with histologic and histochemical pattern of muscle fibers in three biopsies of central core disease, four biopsies of nemaline myopathy, one biopsy of myotubular myopathy, and three biopsies of mitochondrial myopathy. Evidence of collateral reinnervation was obtained only in one biopsy from central core disease. In other biopsies, no structural or ultrastructural abnormality of axis cylinders, myelin, or myoneural junction suggesting denervation were observed. The only relevant change found in centronuclear myopathy and to a lesser extent in nemaline myopathy was an unusual smallness and simplification of motor endings, suggesting delayed or impaired maturation. Muscle fibers strongly reactive for both adenosinetriphosphatase and nicotinamide-adenine dinucleotide diaphorase, observed in central core disease and mitochondrial myopathy, were not associated with increased terminal innervation ratio.

Familial focal loss of cross striations

SummaryTwo patients, a brother and sister, both suffering from congenital generalized muscle weakness with a progressive course are reported. Muscle biopsy revealed areas with loss of cross striations in the muscle fibers, electronmicroscopically presenting complete disorganization of the myofibrils with streaming of the Z discs and absence of mitochondria. Vesicular nuclei with prominent nucleoli were present in these areas. There was a type I fiber prodominance in both cases. The mean diameter of the type I muscle fibers in one of the cases was too small. Motor endplate alterations in this patient gave no evidence of denervation but were suggestive of a delayed development of motor nerves.ZusammenfassungEs werden zwei Patienten beschrieben, Bruder und Schwester, beide an einer kongenitalen, generalisierten, progressiven Muskelschwäche leidend. Die Muskelbiopsie zeigte Zonen mit Verlust der Querstreifung in den Muskelfasern, und bei elektronenmikroskopischer Untersuchung zeigte sich eine totale Unordnung der Myofibrillen mit Strömung der Z-Scheiben und Fehlen von Mitochondrien. In diesen Bezirken fanden sich blasige Kerne mit prominenten Nukleolen. In beiden Fällen zeigte sich ein starkes Überwiegen der Typ-I-Fasern. Der mittlere Durchmesser der Typ-I-Muskelfasern war in einem der Fälle zu gering. Veränderungen in der motorischen Endplatte in diesem Fall zeigten keine Denervierung, sondern deuteten auf eine verzögerte Entwicklung der motorischen Nerven.

Morphologic Study of a Neuromyopathy Caused by Prolonged Chloroquine Treatment

Two muscle biopsies were made in a case of chloroquine neuromyopathy in a 57-year-old woman with discoid lupus erythematosus. Histological study demonstrates widespread granulovacuolar degeneration an

Inflammatory demyelinating polyradiculoneuropathy associated with human immunodeficiency virus infection

SummaryFive patients infected with immunodeficiency virus who were suffering from chronic polyradiculoneuropathy were investigated during evolution of the disease. Four of them were immunodeficient. The prominent neurological feature was distal and symmetrical weakness of the legs. After 5 months only one patient had improved. All patients had an increased protein level in the cerebrospinal fluid and pleocytosis. Electrodiagnostic studies and sural nerve biopsies indicated demyelination. Sural nerve viral cultures, including human immunodeficiency virus, were negative. The presence of circulating anti-peripheral nerve antibodies and of immunoglobulin deposits in nerve biopsy specimens was investigated by immunofluorescence techniques but failed to demonstrate any immunoreactivity.

Pathognomonic aspect of germinoma on CT scan

SummaryBilateral visual impairment, associated with amenorrhea, diabetes insipidus and mental deterioration were observed in a 28 year old woman. CT scan revealed bilateral symmetrical infiltrative processes surrounding the ventricular system, which was confirmed by postmortem examination. Histologicaly the lesion was a germinoma.

Reversible capsulo-tegmental locked-in state as first manifestation of multiple sclerosis.

An 18-year-old girl developed a reversible locked-in state with bilateral ptosis and almost complete ophthalmoplegia. She later presented with a relapsing and remitting course suggestive of multiple sclerosis. Autopsy findings demonstrated bilateral capsular and tegmental demyelinating lesions. In addition to this unusual aetiology, this is the first report with pathological evidence of a locked-in syndrome due to lesions outside the ventral brainstem.