Common Neurological Abnormalities Research Articles

Tick-borne encephalitis in north-eastern Poland in 1997-2001: a retrospective study.

The aim of this study was to characterize the epidemiology and clinical features of tick-borne encephalitis in north-eastern Poland. Clinical and epidemiological data were analysed of patients hospitalized with the diagnosis in the Department of the Infectious Diseases and Neuroinfections of the Medical University in Bialystok in 1997-2001. Tick-borne encephalitis was diagnosed in 152 patients: 51 (34%) presented with meningitis, 89 (59%) with meningoencephalitis and 12 (8%) with meningoencephalomyelitis. Headache (84%) and fever (81%) were the most common symptoms. Meningeal signs were present in 137 patients (90%). Most common neurological abnormalities were: Oppenheim and Babinski signs (74 patients, 49%), ataxia (37, 24%), impaired consciousness (37, 24%) and pareses (16, 10%). Of patients examined, 146 (96%) had raised pleiocytosis, frequently accompanied by high cerebrospinal fluid protein concentration (90%), raised erythrocyte sedimentation rate (65%), peripheral blood leucocytosis (26%) and increased aminotransferase activity (16%). There was only 1 forest worker among the patients. Tick-borne encephalitis remains common in north-eastern Poland but, possibly because of effective vaccination, it has virtually disappeared among forest employees. The diagnosis appears difficult in some cases, as meningeal signs may not be present and laboratory findings may not be suggestive of a viral infection.

Neuropathologic findings after liver transplantation.

Neuropathologic findings are described in 37 patients autopsied after one or more orthotopic liver transplants. Gross or microscopic lesions were observed in almost all patients, including anoxic-ischemic change, hemorrhages and/or infarcts, and opportunistic infections by fungi (most commonly Aspergillus) and rarely viruses (cytomegalovirus). Central pontine and extra-pontine myelinolysis was commonly observed, and appeared to result from severe multifactorial metabolic abnormalities in the perioperative period. Low-grade (microglial nodule) encephalitis without an obvious pathogen was often encountered. Common clinical neurologic abnormalities included encephalopathy, seizures (myoclonic, focal or generalized), obtundation and coma. These were found more commonly than focal findings, but clinical features in a given patient were not uniformly predictive of underlying neuropathologic change.

COCKAYNE SYNDROME (CS) MASQUERADING AS SECKEL SYNDROME (SS)

Our patient presented at 3 months of age with microcephaly, nystagmus, optic atrophy and developmental delay. Birth weight was 2.7 kg. At 11 months he was labeled as SS on the basis of microcephaly, short stature and facial features. Over the next 4 years he developed marked growth failure, mental retardation, ataxia, sunken eyes, photosensitivity, and dental caries. Kyphosis, contractures, lens opacities and long extremities with shortened trunk were present at the time of death at 8 years of age. We identified 132 CS case reports in the world literature comprised of 72 males and 56 females (4 undescribed). Mean (±S.D.) birth weight was 2.9(0±.6)kg. Mean age at death (27 cases) was 14(±8) years. The most common neurologic abnormalities noted were mental retardation (90%), microcephaly (83%), motor disturbance (71%), deafness (47%), intracranial calcification (39%), ventricular dilatation (23%) and peripheral neuropathy (21%). Reported physical manifestations were cachectic dwarfism (91%), characteristic facies (89%), photosensitivity (60%), contractures (52%), long extremities with shortened trunk (43%), dental caries (42%), small or undescended testes (38% of males), kyphosis (21%). Opthalmologic features included retinal pigmentation (51%), optic atrophy (37%), and cataracts or lens opacities (27%). CS is an autosomal recessive leukodystrophy with considerable variation in the expression and progression of clinical manifestations. The constellation of clinical features and the inexorable course of physical and mental deterioration which distinguish CS from SS will be discussed.

Neuropathology of familial erythrophagocytic lymphohistiocytosis: Six cases and review of the literature

The authors report the brain findings in six cases of familial erythrophagocytic lymphohistiocytosis (FEL) and review the literature, focusing primarily on the neurologic and neuropathologic aspects. Clinically, the most common neurologic abnormalities in the six patients were stupor or coma and seizures, with hemiplegia and cranial nerve palsies. Neuropathologic changes were characterized by infiltration of the meninges by lymphocytes and histiocytes and perivascular lymphocytic cuffing and diffuse proliferation of histiocytes in the brain parenchyma. The severity of the brain involvement was variable. In the mildest case there was only meningeal involvement. More severely involved cases showed meningeal and perivascular infiltration of lymphoid cells in the brain. In the most severely involved cases, there was, in addition, diffuse cerebral infiltration by histiocytes accompanied by multifocal necrosis. Some cases showed disproportionately greater involvement of the brain than of the visceral organs. The authors conclude that "meningoencephalitis," histiocytic invasion, and necrotizing brain lesions are characteristic of FEL, although the mechanism of their production is not known. Frequent seizures and disseminated intravascular coagulopathy may be contributory.

The Syndrome of Central Cervical Soft Disk Herniation

Central herniation of soft disk material into the ventral epidural space is a relatively rare cause of cervical myelopathy. A history of significant antecedent trauma or pain is singularly absent in this syndrome. Spastic ataxia of gait and impaired pain and touch sensibility are the common neurological abnormalities. Typically, the myelopathy develops in the presence of a narrowed cervical spinal canal. The myelographic picture is diagnostic but may be misleading as to the exact level of the herniation. Anterior discectomy and fusion have given excellent relief of symptoms in this group of patients.

Helmholtz on Perception: Its Physiology and Development

Marco Mumenthaler, Director der Neurologischen Universitatsklinik Inselspital, Bern. Price, $2.45 (DM 9,80). Pp 383. Georg Thieme Verlag, Stuttgart, Germany. A veritable flood of neurology texts continues to appear and one has difficulty in distinguishing one from another. This soft covered book on neurology by Dr. Mumenthaler is, however, a welcome exception to the usual dreary lot. It is easily carried in a pocket and so can be consulted when needed. The first part has a most excellent review of clinical methods and of the localizing value of abnormal neurological signs. Superb diagrams, tables, and line drawings of common neurological abnormalities encountered at the bedside make this section unique among pocket-sized neurology texts. The diagrams of normal and pathological roentgenographic and electromyographic findings, and of other special tests, are far superior to the often encountered unintelligible photographs. In the second part, neurologic diseases are adequately described, and judiciously selected references are given for those who might want further information, but this should not be necessary for undergraduates. The understanding of neurological disease is again facilitated by drawings and tables, some of which should be of use