Transcranial magnetic stimulation in multiple system and late onset cerebellar atrophies

Central motor conduction time (CMCT) after transcranial magnetic stimulation (TMS) of the cortex, electromyography and nerve conduction velocity were performed in 24 patients with multiple system (MSA) and late onset cerebellar atrophy (LOCA) (often olivopontocerebellar atrophy –OPCA –). CMCT was abnormal in 7 patients with OPCA and one with LOCA. CMCT abnormalities (43% of cases) and increased threshold (68%) were more often found within OPCA group than in another multisystem atrophy and LOCA. Reduction in amplitude of the response after TMS was significantly correlated with cerebral hemispheres's atrophy. Increased threshold was correlated with upper vermal hemisphere atrophy and enlargement of the fourth ventricle. Electrophysiologic signs of mixed peripheral neuropathy were found in 8 patients. TMS abnormalities were not related to peripheral nerve involvement. Marked variation in CMCT suggests heterogeneity in these diseases. However, the percentage of CMCT abnormalities in OPCA group suggests that TMS seems to play a role in the neurophysiological diagnosis of these heterogeneous disorders.     

Electrophysiologic study in benign human botulism type B

Conventional electromyography (EMG) and single fiber EMG (SFEMG) were performed in a 64-year-old diabetic woman with mild type B botulism. The main clinical signs were autonomic dysfunction and cranial nerves paresis. Conventional EMG was normal, except for small changes that were consistent with mild mixed peripheral neuropathy in the lower limbs and were related to diabetes. Repetitive stimulation and results of single stimulus before and after full effort were normal. SFEMG showed increased jitter and impulse blocking in clinically normal muscles. The jitter was frequency dependent and improved at a higher innervation rate. Impulse blocking in potentials with only slightly increased jitter was found. The follow-up showed improvement of the jitter in agreement with clinical recovery. Jitter abnormalities were recorded after 16 weeks, when clinical signs of botulism had been reversed to normal. Motor unit fiber density increased progressively, and on examination at 8 weeks, some potentials showed very high jitter values. Both findings might suggest new endplate formation, perhaps due to ultraterminal sprouting development.     

Protection of the aged substantia nigra of the rat against oxidative damage by (-)-deprenyl.

1. We have studied the effect of (-)-deprenyl on the oxidative damage that the rat substantia nigra suffers during aging. 2. (-)-Deprenyl (2 mg kg-1, three times a week) administered for two months, beginning at 22 months of age, produced a significant increase in tyrosine hydroxylase (TH) activity (2.67 +/- 0.40 and 3.64 +/- 0.38 nmol mg-1 protein h-1 in untreated aged rats and treated aged rats respectively, P < 0.05) and in TH amount (0.072 +/- 0.012 and 0.128 +/- 0.38 absorbance 405 nm in untreated aged and treated aged rats respectively, P < 0.05). 3. The proteins of aged rat substantia nigra showed a significant decrease of carbonyl groups in treated animals compared with saline-injected control rats (136.2 +/- 21.8 and 71.5 +/- 13.2 c.p.m. microgram-1 protein in untreated aged and treated aged rats respectively, P < 0.05). 4. The carbonyl groups measured in TH enzyme showed a statistically significant decrease (42.3%) after (-)-deprenyl treatment (471.4 +/- 73.0 and 271.9 +/- 50.00 c.p.m. in untreated aged and treated aged rats respectively, P < 0.001). 5. All these results suggest that oxidative damage produced during aging is prevented by (-)-deprenyl treatment and could explain the effect of this drug in Parkinson's disease (PD) and other degenerative diseases such as Alzheimer's disease.     

Intrahepatic portal venous hypoplasia in Alagille's syndrome

One new case of arteriohepatic dysplasia (Alagille's syndrome) is reported. The case showed signs of progressive portal hypertension with the liver biopsy revealing evidence of hypoplasia of the intrahepatic portal venous branches without evident cirrhosis, next to the characteristic paucity of intrahepatic bile ducts. This venous abnormality might be responsible for the unfavourable outcome observed in some cases with Alagille's syndrome and corresponds to a severe form of the disease.     

Salvage of the severely injured ear

We present three cases of severe external ear injuries and their management. The first patient's ear, amputated in an automobile accident, was revascularized microsurgically. Replantation, when possible, offers the ultimate result. However, disadvantages include the length of the operation and prolonged hospitalization. The second patient sustained a human bite ear amputation. Salvage consisted of immediate coverage with a vascularized temporoparietal fascial flap and split-thickness skin graft. Revision of the external auditory meatus and postauricular sulcus was required. This technique is relatively simple and reliable when microvascular revascularization is not possible. In the third case the patient's ear suffered a full-thickness burn as part of a larger scalding injury to the patient's body, face and scalp. Acute salvage was accomplished in one stage with a technique similar to that used for the second patient. Secondarily, skin was regrafted to achieve an improved color match and cartilage contour.     

Immunologic dysfunction in Sickle cell anaemia

Selected cellular immune parameters were studied in 20 patients with sickle cell anaemia (SCA). A moderate absolute lymphocytes was observed in SCA patients compared with the control group. There was no significant difference between patients and controls in the mean E-rosette percentage and absolute number. PHA transformation was decreased in 50% of SCA patients, and some of them showed cutaneous anergy. It was suggested that imparied cell-mediated immunity is another factor to be included among defective SCA host-defense mechanism.     

Diagnosing and treating Phthirus pubis palpebrarum

Phthiriasis palpebrarum is an uncommon cause of blepharitis and conjunctivitis and may easily be overlooked. A high index of suspicion and careful examination of the patient's lid margins and eyelashes will lead to the proper diagnosis. Treatment is best accomplished by careful removal of the lice and nits (louse eggs) from the patient's lashes. Local application of a pediculocide such as yellow mercuric oxide N.F. 1% ophthalmic ointment applied twice daily for one week or 0.25% physostigmine (Eserine) ointment applied twice daily for a minimum of ten days, to the lid margins should be considered when the total removal of Phthirus pubis and nits is not possible mechanically. Body hair should be examined for infestation with lice and treated with gamma benzene hexachloride shampoo. This medication should be used with caution in infants, children and pregnant women. Family members, sexual contacts, and close companions should be examined and treated appropriately; clothing, linen and personal items should be disinfected with heat of 50 degrees C for 30 minutes.     

CT of gastric masses: image patterns and a note on potential pitfalls

Computed tomography (CT) was performed in 9 consecutive cases of primary gastric neoplasm. Lesions were surgically or endoscopically proved; cross-sectional images are correlated to specific histopathology in each case. The CT images of leiomyoma and leiomyoblastoma are characterized as models of pure bulging intramural growth resulting in a lunate contrast distribution when imaged in the cross-sectional plane. In 5 cases of lymphoma, distortion of the contrast-filled hollow viscus is relatively consistent. Nodular growth is reflected on the CT image as a series of digitations encroaching on the opacified portion of the gastric lumen. Image pattern recognition, relative to histopathology, is of more than academic interest since endoscopy is frequently unreliable in cases of submucosal and exophytic pathology. Further, biopsy of such lesions is commonly nondiagnostic as a result of random choice of biopsy site or inadequate depth of tissue sample. Additionally, this report includes images of lesions that simulate the primary gastric pathology and may be a source of erroneous interpretation. These include: pseudocyst of the pancreas (2 cases) and enlargement of the left lobe of the liver with encroachment on the gastric lumen (1 case). Image specificity on CT examination is increasingly essential to diagnosis and surgical planning.