Sarcoidosis in a cicatrix: apropos of 5 cases

The authors report 5 cases of sarcoidosis developed on scars. Sarcoidosis may be isolated (1 case); it may precede (1 case) or accompany systemic sarcoidosis, or occur during a relapse (3 cases). Dermatologists who observe cicatricial changes always suspect sarcoidosis. It is therefore important that physicians should examine the scars of their patients with suspected sarcoidosis, since biopsy of these scars is easily performed, confirms the diagnosis and avoids more invasive explorations.     

Current study of Cushing's syndrome, apropos of 3 cases

The authors describe three cases of Cushing' syndrome, due to nodular hyperplasia, simple hyperplasia and adenocarcinoma respectively, and the most useful approaches (dexamethasone, metopirona, insulinic hypoglycemia, cortisol rhythm, catheterism and assessment of urinary free cortisol) for diagnosis and etiology of Cushing's syndrome.     

Emphysematous cystitis: apropos of 2 cases

Emphysematous cystitis is a rare infectious disease of the lower urinary tract. Its reputedly serious prognosis is related to treatment failures revealing ignorance concerning its pathophysiological mechanisms. Two cases of this disease were seen in our department, both in diabetic men over the age of 70 years with a history of prostatism. They presented to the emergency department with complete urinary retention and alteration of the general state and fever. Urine culture isolated Klebsiella pneumoniae in both cases. Treatment consisted of bladder drainage associated with adapted antibiotic therapy and control of diabetes. A suprapubic prostatectomy was subsequently performed in one case and transurethral prostatic resection was performed in the other case. The prognosis of this disease depends on early diagnosis and rapid introduction of effective treatment.     

Sneddon''s syndrome: apropos a case

The case of a 76 year old patient diagnosed of severe dysphagia by familiar oculopharyngeal muscular dystrophy is presented. Central venous catheterization (right internal jugular) was required for parenteral nutrition. A few minutes after catheterization, the patient developed respiratory arrest and coma and later cerebral vascular accident with left flaccid hemiplegia, with the ischemic lesion being focalized in the right parietooccipital region as well as in the left of the posterior fossa were observed. Aneurismatic dissection of the bilateral vertebral artery during lateralization of the head may be a cause of the lesions presented by the patient associated to arterial spasm or not. In central venous catheterization the possible complications derived from the position of the neck may carry must be taken into account.     

Joubert syndrome: a report of 5 cases

OBJECTIVE: To review clinical features, radiological findings and prognosis in Joubert syndrome. MATERIAL AND METHODS: We report 5 children (3 male and 2 female) with the diagnosis of Joubert syndrome by clinical and radiological findings. They were diagnosed in the first year of life, in the Hospital Infantil La Paz (Madrid, Spain), from 1971 to 1996. Three patients have already been published, and here, we report two new cases. RESULTS: Partial absence of the cerebellar vermis, hypotonia and developmental delay were seen in all patients. Other cardinal findings were episodic hyperpnoea (5/5) with periods of apnoea (2/5), abnormal eye movements (2/5) and strabismus (3/5), tongue protrusion (2/5), seizures (1/5), hemifacial spasms (1/5) and occipital meningocele (2/5). Clinical manifestations were first noticed soon after birth. Two patients died in the first 5 years of life, and the rest of the cases actually show severe mental retardation. CONCLUSIONS: Joubert syndrome is a rare and probably underdiagnosed syndrome with bad prognosis. This inherited condition is characterized by agenesis of the cerebellar vermis, mental retardation, hypotonia, episodic hyperpnoea and abnormal eye movements. Additional manifestations have been reported since the original cases were described.     

Buruli ulcers in Burkina Faso: apropos of 6 cases

The authors propose to treat stainless steel dentures by grinding on organosilicon binding followed by 2 polishing procedures: with diamond paste and paste based on ultradispersed aluminum oxide powder. This technology improves the corrosion resistance of dentures and eliminates signs of diseases caused by electrochemical processes in the oral cavity more effectively than basic technology (vulcanite grinding and polishing with GOI paste).     

Thrombotic thrombocytopenic purpura disclosing cancer: apropos of 2 cases

Thrombotic thrombocytopenic purpura (TTP) causes severe haemolytic anaemia, thrombopenia, fever and neurological and renal involvement. Currently five large aetiologic groups have been identified: viral or bacterial infection, drugs, conjunctive tissue diseases, pregnancy and solid tumours. We observed two cases resulting from an adenocarcinoma. In the first case, a 71-year-old man with chronic silicosis, the presenting signs were asthenia, fever, epistaxis with diffus purpura and spontaneous haematomas of the lower limbs. Diagnosis of TTP was based on routine laboratory tests and the patient responded well to fresh frozen plasma. On the 5th day of treatment, haemoglobin level dropped sharply and melana occurred. Upper digestive tract endoscopy revealed a tumoural formation of the antrum-fundic junction and histology examination of the biopsy confirmed the diagnosis of adenocarcinoma. Ten months after gastrectomy the patient was in excellent health with no relapse of the TTP. In the second case, the presenting signs included spontaneous haematomas, rectorrhagia and low grade fever. Microscopic haematuria and renal failure were observed in addition to the biological syndrome of TTP. The patient responded poorly to fresh frozen plasma and packed cell transfusions. Plasma exchange was equally unsuccessful. The disease continued a fulminant course and the diagnosis of adenocarcinoma located in a pulmonary lymph nodes was made at autopsy. These rare cases of TTP caused by cancer emphasize the importance of a thorough aetiological research. Plasma exchange has been shown to be effective but mortality at 1 year approximately 85% in cancer related cases. Early diagnosis and specific anti-cancer therapy might improve prognosis. We report our personal experience with 16 other similar cases.     

Acute schistosomiasis or Katayama syndrome: apropos of 2 mini-epidemics

Schistosomiasis, or bilharziasis, is often oligosymptomatic but there is also an acute form, the Katayama syndrome. Observation of two groups of travelers who swam in the same lake near Banfora in western Burkina Faso, separated by an interval of one year, illustrates the high infection rate of Schistosoma mansoni (100% of swimmers, of whom 82% were symptomatic and 55% presented with fever). The incubation period was 4 to 6 weeks after exposure. 10 of the 11 subjects had eosinophilia (> or = 500/mm3) and IFAT serology was positive in all tested subjects. More than one year after exposure, one traveler (untreated) presented with a neurological deficit due to acute schistosomal myelitis. The diagnosis of schistosomiasis in a patient requires a search for the disease among his fellow travelers, even if they are asymptomatic.     

Leprosy tests: diagnostic problems (apropos of 2 cases)

Two cases of reactional leprosy leading to wrong diagnosis are reported. The first one concerns a reversal reaction predominantly neuritic, initially taken for polyarthritis. The second one concerns an erythema nodosum leprosum with extensive cutaneous necrosis (Lucio's phenomenon or ulcerative lazarine leprosy). Main aspects and mechanisms of leprosy reactional states are reviewed. It is emphasized that errors or delays in diagnosis are often caused by failing to recognize cutaneous or neuritic symptoms.     

Treatment of sudden deafness apropos of 447 cases

Tibial nerve and S1 dermatome somatosensory evoked potentials (SSEPs) were recorded before and after iohexol lumbar myelography in order to evaluate possible neurotoxic effects of this contrast medium. No significant change in SSEP latencies nor amplitudes was noted after iohexol myelography, supporting the low neurotoxic profile of this contrast agent. Results were compared to those of a control group of patients before and after lumbar puncture (LP), without injection of contrast agent. In this group also no significant change in SSEP components was found, indicating that a preceding LP does not affect this electrophysiological examination.     

Inhalation burns: apropos of 198 cases. Incidence of laryngotracheal involvement

Inhalation burns and laryngotracheal involvement were studied in a retrospective series of 635 patients hospitalized for skin burns at the Center for Burn Treatment from January 1993 to January 1997. Inhalation burns were observed in 31.1% of the cases. Exclusive laryngeal involvement occurred in 19.6% of the inhalation burns. Both tracheobronchial and laryngeal burns were observed in 27.2%. Patients with inhalation burns also had facial burns (90.9%) and extensive (> 50%) or severe (UBS > 200) skin burns in 39.8% and 29.7% of the cases respectively. Mortality of skin burns was increased six-fold to 19.1% in patients who also had inhalation burns. Intubation was used alone in 60.1% and was followed by tracheotomy in 27.2%. The decision for tracheotomy was essentially based on the probable duration of ventilatory assistance. Tracheotomy was required in case of severe inhalation burns and the predictable duration of intubation was over 8 days. Laryngotracheal stenosis occurring after inhalation burns is complex and extensive, with great variability over time. Laryngotracheal calibration is indicated as first intention therapy.     

Rendu-Osler disease: apropos of 50 cases followed at the Gustave-Roussy Institute

Rendu-Osler disease is an hereditary disease due to a predominantly antosomic heredity. The statistics given for 50 cases include 27 males 7 p. 100 of which had no familial antecedents. Clinically, Rendu-Osler disease begins in adulthood (44 p. 100) and often in late adulthood. In fact, it does not become really severe until about the age of 50 or 60. Epistaxis is the most frequent accident and proves fatal in more than 10 p. 100 of cases. Treatment of the nasal symptoms, whether this involves surgical excision followed by grafting or plesiocurietherapy using irridium wires, is very disappointing. The future alone will prove whether embolization gives more permanent results.     

Cervical ribs. Therapeutic deliberations apropos of 10 cases

The authors report ten cases of cervical ribs operated upon in 7 patients. The predominantly neurological symptoms and signs were associated with vascular problems in 3 cases, with intermittent compression of the sub-clavian vessels. Treatment consisted of resection of the cervical rib and the first rib via an extra-pleural axillary approach using the technique described by Roos. It gave 10 good results with a follow-up of 6 months to 3 years. The authors emphasise the fact that this pathology falls within the context of compressive syndromes of the root of the upper limb or thoracic outlet syndrome. Such a conception justifies associated resection of the first rib which should give better long term results than simple resection of the extra rib.     

Anosognosia in parietal lobe syndrome

Patients with right parietal lesions often deny their paralysis (anosognosia), but do they have "tacit" knowledge of their paralysis? I devised three novel tests to explore this. First, the patients were given a choice between a bimanual task (e.g., tying shoe laces) vs a unimanual one (e.g., threading a bolt). They chose the former on 17 of 18 trials and, surprisingly, showed no frustration or learning despite repeated failed attempts. I conclude that they have no tacit knowledge of paralysis (or, if such knowledge exists, it is not available for this particular task). Second, I used a "virtual reality box" to convey the optical illusion to the patient that she was moving her paralyzed left hand up and down to the rhythm of a metronome, and yet she showed no sign of surprise. Third, I irrigated patient BM's left ear canal with cold water, a procedure that is known to shift that patient's spatial frame of reference by stimulating the vestibular system. Surprisingly, this allowed her "repressed" memory of the paralysis to come to the surface; she said she had been paralyzed continuously for several days. I suggest that the vestibular stimulation produces these remarkable effects by mimicking REM sleep. These patients also employ a whole arsenal of grossly exaggerated Freudian "defense mechanisms" to account for their paralysis. To explain this, I propose that in normal individuals the left hemisphere ordinarily deals with small, local anomalies by trying to impose consistency but, when the anomaly exceeds threshold, an interaction with the right hemisphere forces a "paradigm shift." A failure of this process, in patients with right hemisphere damage, might partially account for anosognosia. Finally, I present a new conceptual framework that may help link several psychological and neurological phenomena such as Freudian defense mechanisms, vestibular stimulation, anosognosia, memory repression, visual illusions, anterograde amnesia, REM sleep, dreaming, and humor.