Critical illness myopathy and neuropathy.

Muscle wasting and paralysis are common complications in Intensive Care Unit (ICU) patients, where critical illness polyneuropathy (CIP) and critical illness myopathy (CIM), alone or in combination (CIP/CIM), are the commonest causes. CIP is an acute axonal sensory-motor polyneuropathy usually suspected in ICU patients who, after a period of days or weeks, cannot be weaned from the ventilator despite the absence of pulmonary or cardiac causes of respiratory failure, or because they suffer from various degrees of limb weakness. CIM is an acute primary myopathy with a continuum of myopathic findings, from myopathies with pure functional impairment and normal histology to myopathies with atrophy and necrosis. The true incidence of CIM is unknown, because neither the diagnosis of CIM nor the differential diagnosis between CIP and CIM in the ICU are easy, and requires specialized neurophysiological methods or biopsy investigations in addition to conventional nerve conduction studies and needle electromyography. When these methods are used, CIM is as frequent as or more frequent than CIP. Failed weaning of patients from the ventilator, inappropriate evaluations of comatose patients and prolonged disability after ICU discharge are common consequences of CIP/CIM. Recent data indicate that CIM has a better prognosis than CIP, and differential diagnosis is therefore important to predict long term outcome in ICU patients. Bioenergetic failure is thought to be a relevant pathophysiological mechanism explaining both CIP/CIM and multi-organ failure. Indeed, CIP/CIM itself should be considered as the failure of the peripheral nervous-muscular system.

Pretibial myxoedema associated with Hashimoto's thyroiditis.

Pretibial myxoedema is a cutaneous mucinosis typically associated with Graves' disease, although it may also develop in subjects with non-thyrotoxic thyroid pathologies. This report presents a rare case of pretibial myxoedema occurring in a 58-year-old woman with biopsy-proven Hashimoto's thyroiditis. The hypothetical pathogenetic link between the two disorders is discussed with particular attention to the role of thyroid stimulating hormone receptor antibodies.

Successful treatment of pustulotic arthro-osteitis (Sonozaki syndrome) with systemic cyclosporin.

Pustulotic arthro-osteitis, first described by Sonozaki, is a relatively rare disorder, the prevalence of which is probably underestimated in dermatological literature; its early recognition can prevent misdiagnosis, unnecessary surgery, and the use of prolonged and ineffective antibiotic treatment.

Multiple familial basal cell carcinomas including a case of segmental manifestation.

BACKGROUND: A tendency to develop multiple basal cell carcinomas at an early age is a characteristic feature of some rare hereditary disorders; moreover, multiple basal cell carcinomas are sometimes observed as a corollary of familial basaloid follicular hamartomas or familial multiple trichoepitheliomas. OBSERVATION: We report 3 cases of multiple basal cell carcinomas involving 3 successive generations of a family, with a segmental manifestation of lesions in one of these patients. No additional cutaneous or extracutaneous anomalies were found. CONCLUSIONS: We hypothesize that a gene mutation may have caused the tumors observed in this family as an autosomal dominant trait. The segmental arrangement of tumors may reflect loss of heterozygosity: at an early stage of embryogenesis, a postzygotic mutation would give rise to a population of cells either homozygous or hemizygous for the underlying gene. The segmental arrangement following the lines of Blaschko would visualize the dorsoventral proliferation of a cell clone characterized by loss of the corresponding normal allele.

Subcorneal pustular dermatosis and IgA lambda myeloma: a uncommon association but probably not coincidental.

The present report deals with a case of subcorneal pustular dermatosis (SCPD) associated with IgAlambda myeloma and reviews the literature for similar cases. Three relevant points arise from this case: the association of the dermatosis with an IgAlambda myeloma that, as far as we know, has been described only three times previously; the longest period of time between the onset of the dermatosis and that of the myelopathy observed up to now; the good therapeutic response to etretinate, useful in the management of severe recalcitrant forms of SCPD.

Skin pathology findings in a cohort of 1500 adult and elderly subjects.

BACKGROUND: No extensive studies are available in the literature on the eventual skin pathology induced by neurologic or systemic diseases in elderly individuals. Other factors, such as health and hygiene, socioeconomic status, and climate can also play an important role. METHODS: Fifteen-hundred subjects (886 women and 614 men; mean age, 67.8 years; range, 39-90 years) were admitted to the Department of Geriatrics at the Oasi Institute between 1992 and 1997; all these subjects were carefully evaluated from a dermatologic point of view. Each subject underwent specialist examinations, routine blood analyses, thoracic X-rays, cerebral computerized tomography (CT) scan, and magnetic resonance imaging (MRI) when appropriate. A group of subjects without significant neurologic or systemic disease, comprising 116 women and 60 men (mean age, 64.5 years; range, range, 40-90 years), was selected and used as a normal control group. Subsequently, our attention was focused on the eventual presence of the following neurologic diseases: Alzheimer-type dementia, vascular dementia, mixed-type dementia, subcortical dementia, Parkinson's disease, vascular brain disease, hemiplegia, etc. Thus, different subgroups were formed on the basis of such diagnostic categories and the frequency of skin pathology in each subgroup was evaluated. RESULTS: Of the 1500 subjects, 1439 stated that they had never been affected by dermatologic disease. No statistically significant difference in frequency of skin pathology was found between normal controls and the different patient subgroups. Unsuspected and singular dermatoses were found, however, such as paraneoplastic syndromes, idiopathic tripe palms, white fibrous papulosis of the neck as an expression of photoaging, conditions induced by former popular traditions of Sicilian culture (anetoderma secondary to the application of Hirudo medicinalis and erythema ab igne), pigmented dermatoses never described before in Italy (prurigo pigmentosa and friction amyloidosis), and nail abnormalities (atypical half-and-half nail, and dyschromic nail changes in multiple system atrophy and in hemiplegia). CONCLUSIONS: The dermatologic screening performed in 1500 patients revealed several unexpected diagnoses and some original observations. Some rare dermatoses were described and certain hypotheses were suggested to explain the peculiar dyschromic changes of the fingernails in multiple system atrophy, the atypical cases of half-and-half nail, and the so-called idiopathic tripe palms associated with psoriasis.

Critical illness myopathy and neuropathy.

BACKGROUND: Critically ill patients may develop muscle weakness or paralysis during the course of sepsis and multiple-organ failure. We studied peripheral nerve and muscle disorders (NMD) in comatose patients. METHOD: Comatose patients who developed paralysis associated with absent deep-tendon reflexes had electroneuromyography (ENMG) and muscle-nerve biopsy specimens taken. Onset and duration of sepsis, multiple-organ dysfunction and failure, biochemical alterations, and drugs potentially interfering with nerve-muscle function were recorded. FINDINGS: 24 patients became quadriparetic or quadriplegic; muscle changes were found in 23. Axonal neuropathy was found in eight of 22 patients examined. All patients had prolonged sepsis and multiple-organ dysfunction, but only 14 had multiple-organ failure. Drugs such as steroids, neuromuscular-blocking agents, and aminoglycosides were not responsible for paresis, and the part played by hyperglycaemia and hypoalbuminaemia is uncertain. Attending physicians predicted a fatal outcome in all cases, although six of seven survivors fully recovered within 115-210 days from the onset of paralysis. INTERPRETATION: Comatose patients may become completely paralysed because of NMD. The diagnosis is important to avoid unnecessary investigations and unreasonably pessimistic prognosis. ENMG is essential for the diagnosis and for planning further clinical management. Biopsy needs to be done only when it is necessary to properly classify NMD.

[Oleoma of the knee. A clinical case]. / Oleoma del ginocchio. Descrizione di un caso clinico.

Authors describe clinical and anatomo-pathological features of a case of oleoma of the left knee, repaired with success through the rotation of a muscular flap of lateral gastrocnemius and application of dermo-epidermal graft. Postoperative follow-up was regular; prompt healing and morpho-functional restoration of involved structures was complete.

Monitoring of somatosensory evoked potentials during carotid endarterectomy: relationship with different haemodynamic parameters and clinical outcome.

The authors studied the changes of Somatosensory Evoked Potentials (SEPs) performed in 241 anaesthetised patients during 264 carotid endarterectomies (CEs). SEP responses were considered significantly modified when the central conduction time was greater than 1 ms and/or when the amplitude of the complex N20-P25 decreased by at least 50%. Both CCT and N20-P25 have been correlated with different parameters, including the presence or absence of preoperative neurological deficits, the type of general anaesthesia, the status of the contralateral and the ipsilateral carotid artery, stump pressure, the use of an intraluminal shunt and the perioperative results. After carotid cross-clamping SEP responses were within the normal range in 236 CEs (89%), and abnormal in 28 (11%). A shunt was inserted 23 times in 264 (9%) cases. None of the patients operated on in this series experienced a permanent neurological deficit; there were three (1.1%) transient deficits (two Rinds and one TIA) and two deaths from non-neurological causes. Only one of the transient deficits was present when the patient woke from the anaesthetic and this event was predicted by significant modification of the SEP which did not reverse after removal of the clamps (a shunt was not used). None of the patients in our series who did not present significant modifications of SEPs during the operation had any postoperative neurological deficit. The authors conclude that SEP recording is a highly reliable and objective method for continuous monitoring of brain function during CE.

Influence of contralateral carotid artery on neurologic outcome after carotid endarterectomy.

The discrepancy in the literature about the immediate results after carotid endarterectomy in the presence of occluded or seriously stenotic contralateral carotid artery, has encouraged a retrospective study on 230 patients subjected to 255 carotid endarterectomies. 63 operations (group 1) were carried out in the presence of occluded contralateral carotid artery (40 operations) or of seriously stenotic contralateral carotid artery (23 operations). In the remaining 192 operations (group 2) the contralateral carotid artery was normal or not significantly stenotic (less than 60% of diameter reduction). The indication for surgery and age and sex distribution were similar in the two groups. No operations were performed during acute stroke. Patients with previous stroke underwent surgery only after at least 1 month from the onset of symptoms, in stable neurological conditions. All the patients were operated on under general anesthesia and with systemic heparinization; the indication for intraluminal shunt was made on the basis of clinical evaluation, of back pressure value and, in 106 operated cases of somatosensory cortical evoked potentials. At the end of every operation, ultrasonographic and/or angiographic instrumental controls were carried out. No statistically significant difference was evidenced in the incidence in groups 1 and 2 of postoperative transitory neurological insufficiency (both 3.2%, P greater than 0.8), of permanent neurological insufficiency (0% and 1%, P greater than 0.9), of mortality because of neurological (3.2% and 1%, P greater than 0.5) and non neurological causes (1.6% and 0%, P greater than 0.1).(ABSTRACT TRUNCATED AT 250 WORDS)