The syndrome of diabetic amyotrophy.

Changes in the electromyograms and motor nerve conduction velocities in 12 patients with diabetic amyotrophy suggested mild distal and moderate proximal neuropathy in the lower limbs. Histological and histochemical findings in the vastus medialis muscles were consistent with denervation. Electron microscopical examination of the vastus medialis muscles in 6 patients revealed myofibrillar degeneration. One patient had abnormal mitochondria and tubular aggregates. The basement membranes of the intramuscular capillaries were thickened in all but 1 patient. Histochemical staining of the myoneural junctions showed changes consistent with degeneration and regeneration. We conclude that diabetic amyotrophy is a distinct clinical entity and is secondary to metabolic derangement rather than diabetic microangiopathy.     

Hysteria 100 years later

Is it true that hysteria we see today is no longer that described at the end of the last century? Is it true that Charcot's work on hysteria ended in failure? Contrary to currently accepted opinion, it can be demonstrated not only that Charcot truly laid the foundation for the psychological theory of hysteria but that his explanation of the mechanism of conversion forms the basis for one of the most effective therapies. Furthermore the major streams of thought in psychopathology have come to complete Charcot's point of view and in no way contradict it. A general view of neurosis can thus be distinguished, while not forgetting that these complementary points of view are based on logics of action irreducibles to each other. The interest shown by Charcot in cultural phenomena, such as ecstasy or demoniacal possession, even introduces to an anthropological point of view which situates the hysterical symptom within the more general framework of a universal modality of action.     

Kleine-Levin syndrome 15 years later

A Chinese man with Kleine-Levin syndrome showed evidence of hypothalamic disturbance and damage to the limbic system. He has been free from symptoms of hypersomnia and hyperphagia for 15 years, but now suffers from ejaculatory impotence which is discussed in the light of aetiological theories of this puzzling disorder.     

Electrophysiological study of amyotrophy associated with acute asthma (asthmatic amyotrophy).

A girl developed acute amyotrophy of the left arm five days after onset of an acute attack of bronchial asthma. Slow improvement in muscle power began nine months later. There was severe denervation with preservation of sensation and normal motor nerve conduction velocities when these were measurable. The patient is similar to 10 patients reported by Hopkins (1974).     

Distinctive pathologic findings in proximal diabetic neuropathy (diabetic amyotrophy)

OBJECTIVE: To investigate the pathogenesis of proximal diabetic neuropathy (PDN) with nerve and muscle biopsies. BACKGROUND: Recent evidence suggests that nerve ischemia secondary to immune-mediated vasculopathy rather than diabetic microangiopathy may be responsible for PDN.Method:- Fifteen patients with PDN and two diabetic controls underwent nerve and muscle biopsy and clinical, electrophysiologic, and laboratory evaluation. There were eight men and seven women between 49 and 79 years of age with type II diabetes. All had progressive, painful, asymmetric, proximal weakness with duration of 5 weeks to 12 months. None had evidence of systemic autoimmune disorder. RESULTS: Four patients showed the distinctive findings of polymorphonuclear small-vessel vasculitis affecting epineurial vessels with transmural infiltration of postcapillary venules with polymorphonuclear leukocytes. Immunoglobulin M (IgM) deposits were found along the endothelium and intramurally in affected vessels. IgM staining was seen in the subperineurial space and in the endoneurium. Activated complement deposition was seen along endothelium of small vessels. Three of these four patients were evaluated within 6 seeks of onset of PDN, and the fourth patient during acute flare of PDN 6 months after the initial onset. Six patients showed "perivasculitis" with mononuclear cell infiltrates around small epineurial vessels without vasculitis (fibrinoid necrosis or transmural inflammation). One patient showed recanalized vessels with transmural lymphocytes without fibrinoid necrosis, possibly suggesting healed vasculitis. CONCLUSION: These distinctive pathologic findings support that proximal diabetic neuropathy has an immune-mediated inflammatory basis and suggest that polymorphonuclear vasculitis with immune complex and complement deposition may be the primary event in the acute phase of proximal diabetic neuropathy.     

Acute postasthmatic amyotrophy (Hopkins' syndrome)

Acute postasthmatic amyotrophy is a rare condition, previously reported in only 26 cases. It is characterized by a sudden onset of a flaccid paralysis of an arm or a leg with completely preserved sensibility, about 1 week after an asthmatic attack. The cause is probably due to a lesion of the anterior horn of the spinal cord, but evidence indicates a more widespread pathological process. The etiology is unknown. but infectious or immunological mechanisms are likely. © 1994 John Wiley & Sons, Inc.     

Sensory nerve involvement in X-linked bulbospinal amyotrophy (Kennedy syndrome). Contributions of electrophysiologic and histologic data

Two new cases of X-chromosome linked bulbospinal muscular atrophy associated with gynecomastia (described by Kennedy in 1968) are reported. In one patient, an electrophysiological study and a muscle nerve biopsy were performed. Motor nerve conduction studies were normal but sensory action potentials were small or unrecordable in the absence of clinical sensory loss. Superficial peroneal nerve biopsy showed axonal lesions. These findings suggest that this disease is not purely due to degeneration of spinal motor neurons and is also associated with an axonopathy.     

3 cases of scapulo-peroneal neurogenic amyotrophy (Dawidenkow's syndrome). Nosological situation in relation to Charcot-Marie-Tooth disease

Three cases of neuropathic scapulo-peroneal syndrome are reported. One case was of the hypertrophic type, two were of the neuronal type. The nosological situation of the Dawidenkow syndrome is discussed. Cases of scapulo-peroneal amyotrophy with peripheral neuropathy should be regarded as a topographical variety of Charcot-Marie-Tooth disease.     

The Denervated Muscle: 45 years later

Forty-five years after its publication, Ernest Gutmann's book, The Denervated Muscle, still stands as a landmark publication. It summarized the state of knowledge of the time and introduced many new researches that were continuing at the Institute of Physiology in Prague. At the time, the response of a muscle to denervation was viewed primarily through the lens of the neurotrophic theory. Advancements in our understanding of neurotrophic effects and mechanisms would now call into question some of the hypotheses and interpretations presented in the book, but many of the research findings have stood the test of time. This review will cover some of the questions asked and data presented in this book, and will place them into the context of contemporary muscle biology.     

Acute shoulder-girdle neuralgic amyotrophy (Parsonage-Turner syndrome with saphenous nerve involvement

Parsonage-Turner syndrome (PTS) is a relatively rare cause of upper extremity weakness and pain. There is currently no effective treatment for PTS although corticosteroids are recommended. Here we report the case of a man with acute PTS and exceptional involvement of both saphenous nerves, advantageously treated with intravenous immunoglobulin.     

The cloak of competence: years later.

A sample of the persons in Edgerton's 1960-1961 study, The Cloak of Competence, was revisited in 1972-1973. The life circumstances of many of these persons had improved, but predictions of the direction and nature of community adjustment in a number of cases proved to be inaccurate. We discussed the issue of diversity in these cases and factors which make prediction problematic, such as environmental variables (as opposed to personal ones) and the effects of time on individual adaptation. Caution was suggested in looking at short-term adaptation without considering these factors. The importance of recognizing the individual's own definition of success was also stressed.     

Cesare Lombroso and epilepsy 100 years later: an unabridged report of his original transactions

Cesare Lombroso (1835-1909) still represents one of the most famous and, at the same time, controversial figures of neuropsychiatry. His idea of the "atavist" criminal, prisoner of his/her biologic inheritance, became extremely popular in Western countries. Unfortunately, Lombroso's theory of a strict connection between epilepsy and the criminal personality exerted a long-lasting negative influence on both medical and public opinion, and strongly contributed to the stigmatization of patients with epilepsy. In this report, we aim to present the complete and unabridged series of passages of Cesare Lombroso's works, published only in the Italian language, discussing the issue of epilepsy and crime. All original Italian quotations are offered and an English translation is provided. Although we believe that the medical profession must be well aware of the "evil" distortions generated by Lombrosian theories, which clearly emerge from his original transactions, any comment is deliberately avoided because, in our opinion, people and their ideas must be judged in accordance with the historical period to which they belonged.     

Chronic spinal amyotrophy involving the upper limbs in young adults (O'Sullivan and McLeod syndrome). MRI study of the cervical spinal cord

In 5 cases of sporadic spinal muscular atrophy in young adults the muscular atrophy was localized in the hands and forearms. Age at onset ranged from 7 to 20 years. The duration of the disease was less than 5 years in 3 patients and more than 15 years in 2. Electromyography showed neurogenic anomalies in all wasted muscles, denervation potentials being also present in the proximal muscles of the upper limbs and distal muscles of the lower limbs in 2 patients. Motor nerve conduction velocity and sensory nerve action potentials were normal. Advantages of a topographic classification of the distal form of chronic spinal muscular atrophy are discussed. Spinal cord MRI was performed in 4 patients with a 0.5 Tesla superconducting magnet using surface coils, using a T1-weighted spin-echo technique (ET 26 ms; RT 500 ms) and a T2-weighted spin-echo technique (ET 90, 180 ms; 2,000 ms). With the T1 technique, axial MRI sections 7 to 9 mm thick of spinal cord showed a normal image between C1 and C4, and a flattened image between C5 and T1. This was considered as a possible spinal cord segmental atrophy.