Pseudohypertrophic myopathy in cysticerosis.

A patient with diffuse involvement of the central nervous system and pseudohypertrophic muscular changes induced by cysticerci is described. Electromyographic and pathologic changes are reported for the first time. Electromyographic examination demonstrated numerous short-duration, low-amplitude motor unit potentials in proximal muscles. Biopsy showed swelling of muscle fibers, fiber atrophy with fibrosis, and cellular infiltration separate from inflammatory exudate surrounding numerous cysts.

Neurological consequences of protein and protein-calorie undernutrition.

Malnutrition is a worldwide problem of enormous magnitude. The growth of the central nervous system in human beings is retarded in case of malnutrition in the very early part of life. Likewise, the peripheral nerves in infants and children and young growing animals appear susceptible to nutritional deprivation including protein as well as protein-calorie deficiency. Motor weakness, hypotonia, and hyporeflexia in infants and children are the essential clinical neurological signs in protein-calorie malnutrition (PCM). Motor and sensory nerve conduction are significantly impaired in children with PCM as well as in animals subjected to protein or protein-calorie deficiency. Histological studies have revealed reduced diameter of myelinated nerve fibers, retardation of myelination, segmental demyelination and remyelination, axonal degeneration, and shortened longitudinal growth of internodes. Diffusion barrier by perineurium may be broken. There is reduction in myelin lipids and impaired synthesis of myelin as shown by the biochemical and radioisotope incorporation studies. Presence of cholesterol esters in the biochemical synthesis of nerves suggests degeneration changes. Experimental studies have revealed that most effects of PCM on peripheral nerves can be reversed by nutritional rehabilitation, although complete recovery in the sensory nerve action potential, fiber size of dorsal nerve roots, and myelin-specific lipids does not occur. Skeletal muscle also shows many changes including muscle fiber atrophy, reduction in duration and amplitude of motor unit potentials, and/or fibrillation on electromyography (EMG) and biochemical estimation of muscle enzymes. These changes may be the reflection of a direct effect of PCM on muscles or secondary to the abnormal structural or biochemical changes in the peripheral nerves. PCM affects the central nervous system, especially the neuropsychological functions, in a lasting manner. Learning deficits and impairment of manual dexterity are the most obtrusive features. Neurotransmitter abnormalities and maturation lag in electroencephalogram have been demonstrated in experimental animals. Spinal cord dysfunction sometimes manifests overtly as clinical myelopathy. Degenerative changes in the cerebellum have been noted.

Phrenic neuropathy in arsenic poisoning.

A patient presented with acute arsenic neuropathy with asymmetric bilateral phrenic nerve involvement. The clinical and roentgenographic observations of phrenic nerve dysfunction were confirmed by prolonged phrenic nerve conduction time. The patient made a significant recovery with d-penicillamine therapy.

Ataxia telangiectasia in identical twins: unusual features.

The cases of identical twins with ataxia telangiectasia, early intellectual impairment and progressive spasticity are reported. Immunological tests revealed reduced levels of serum and salivary IgA, increased B cells, reduced T cells and raised alpha-fetoprotein. CT scan performed in one of the twins was normal. The pathogenesis of the spasticity is discussed.

Protein energy malnutrition and the nervous system.

Protein-energy malnutrition (PEM), a natural ramification of poverty, continues to be a perennial source of concern to a large segment of the world population. The developing nervous system of a child is specially vulnerable to deprivations in nurture. Peripheral nerve and muscle derangements are clinically evident by weakness, hypotonia and hyporeflexia in accordance with severity and duration of PEM. Motor and sensory nerve conduction studies exhibit significant abnormalities and often furnish useful and ominous correlation with grades of PEM. The human sural nerve histology in cases of severe PEM is characterized by persistence of small myelinated fibres, striking failure of internodal elongation and significant segmental demyelination. Young rhesus monkeys are ideal experimental PEM models and they show myopathic EMG changes amenable to rehabilitation. Muscle pathology comprises obliteration of cross-striations, streaming of Z bands, increased interfibrillary spaces, mitochondriomegaly and small-for-age fibres. Radioisotope assays reveal anomalous incorporation into various nerve and muscle constituents. Central nervous system, specially the neuropsychological functions are affected in a lasting manner. Learning deficits, behavioural problems and manual indexterity are most obtrusive features.

Pathology and time relationship of peripheral nerve changes in experimental diabetes.

Structural changes have been studied in peripheral nerves in streptozotocin-induced diabetic monkeys. Teased single nerve fibre preparations were most informative. Abnormalities were present in 32 of 58 nerves examined from 15 diabetic monkeys with varying degree of hyperglycaemia. The earliest change was an increase in the gap at nodes of Ranvier, seen 12 weeks after the diabetic state had been established. Well marked segmental demyelination was seen in distal nerves at 14-16 weeks. Later similar changes were seen in proximal, larger nerves. Evidence of remyelination was present at a later date. Wallerian degneration was seen in only 4 nerves. Changes in the myelin sheath were more prominant than axonal abnormalities at all times. There was no abnormality in the vasa nervorum and only a mild increase in endoneurial and perineurial fibrous tissue. A direct correlation was present between the extent and degree of pathology and both severity as well as duration of hyperglycaemia.

Vanishing CT lesions in epilepsy.

Experience with computed tomography (CT) in patients with epilepsy has drawn attention to specific type of cerebral lesions widely prevalent in this part of the world. The lesions disappear spontaneously while patients receive only anticonvulsant therapy. The exact nature of these lesions is not yet understood. One-hundred and twenty-two such patients (M 77; F 45) were studied. Their age ranged from 3 to 57 (mean 21.8 +/- 12.7) years. Seizures were partial motor in 86 (70.5%), generalised tonic clonic in 31 (25.4%), complex partial in 4 and atonic in one case. Postictal hemiparesis was seen in 6 patients who completely recovered within 5 min to 7 days. One hundred patients underwent EEG examination. It was abnormal in 84 cases; 40 patients showed localisation which correlated with CT findings. Plain CT showed low attenuation lesions in all cases. Morphology on contrast enhancement was ring lesion in 50 (40.9%), disc lesion in 47 (38.5%), target lesion in 3 (2.5%), mixed lesion in 2 (0.8%), no enhancement in 18 (14.8%) and a speck with oedema in 2 (0.8%) cases. All lesions were cerebral, present at cortical-subcortical junction. The first follow-up scan was performed in 78 (63.9%) patients within 6-12 weeks. It showed complete disappearance of the lesion in 47 (60.3%) cases. Reduction in size of the lesion and surrounding oedema was observed in 24 (30.8%) cases. The follow-up scans in these 24 cases revealed disappearance of lesion in 11 cases and almost complete resolution of the lesion with a remaining speck in 13 cases. Cerebrospinal fluid (CSF) was normal in 95 out of 100 cases in which it was performed.(ABSTRACT TRUNCATED AT 250 WORDS)

Neurological complications of acute haemorrhagic conjunctivitis.

Forty four cases of the neurological complications of acute haemorrhagic conjunctivitis (AHC) seen in India during 1981 epidemic are reported. The disease predominantly affected adult males. The preceding attack of AHC, a latent period, prodromal symptoms of fever, myalgia and root pains followed by acute onset of lower motor neurone paralysis of limbs and/or cranial nerves formed the classical picture of neurological involvement. The recovery was poor and nearly half of the patients remained severely handicapped. Electrophysiological studies showed early appearance of widespread fibrillations and fasciculations, large polyphasic potentials of increased amplitude and reduced interference pattern. Nerve conduction studies were normal in most of the cases. Cerebrospinal fluid (CSF) examination revealed lymphocytic pleocytosis and rise in protein content. Significant antibody titres against enterovirus type 70 (EV 70) were demonstrated in the serum and the CSF. HLA studies showed low occurrence of A2 and B15 HLA antigens. Muscle biopsies revealed neurogenic atrophy and sural nerve biopsies were histologically unremarkable. The similarities of this disease with poliomyelitis and its pathogenesis are discussed.

A case control study and one year follow-up of registered epilepsy cases in a resettlement colony of North India, a developing tropical country.

A population-based case control study and monthly follow-up of 121 registered epilepsy cases was conducted during 1995-1997 in a resettlement colony of Chandigarh, India. History of various tentative risk factors, e.g. trauma, febrile seizures, family history of seizures, alcohol intake and other possible causes was elicited. An age- and sex-matched control was selected from the neighbouring families for each case. A discordant pair analysis was done for matched case/controls. History of head injury, febrile seizures and developmental delay was observed exclusively in cases (none present in controls). Odds for epilepsy were higher among people who had positive family history (O.R.= 2.1, chi2 = 5.5, C.I. = 1.1-4.3). All cases were followed up and interviewed for history of seizures and drug intake. Fourteen cases could not be followed up completely. Ninety-four (88%) of the remaining 107 cases did not have any seizures during the follow-up. Of them, 70 (75%) patients were not on medication, 13 patients were on phenytoin and 11 patients received phenobarbitone. Thirteen cases reported seizures during the follow-up. Four patients out of the latter had mental retardation and were not on medication. Cumulative incidence of epilepsy was estimated to be 0.6/1000 person-year exposure.

Cysticerciasis and epilepsy: a clinical and serological study.

Study of 1,038 randomly selected cases of epilepsy in Chandigarh showed the cysticercus haemagglutination test to be a useful adjunct in the diagnosis of cysticerciasis as an aetiological factor. It was positive in 25.7% of epilepsy cases but in only 2% of healthy controls. The rate of seropositivity was higher in focal than in generalized epilepsy although the difference was not statistically significant. Incidence of seropositivity was about equal in males and females but did not appear to be related to the duration of epilepsy.

Acute cerebellar ataxia in enteric fever.

Acute cerebellar ataxia as an isolated neurological manifestation of enteric fever is very rare. Three cases of acute cerebellar ataxia associated with enteric fever are reported. The diagnosis of enteric fever was confirmed by positive blood culture, strongly positive Widal test and rising antibody titres. The major clinical features were rapid development of gait ataxia, limb ataxia and dysarthria. None of the patients had altered sensorium. The cerebellar involvement was noticed on the second or third day of fever which progressed for one to two days. The symptoms remained static for one to two weeks and thereafter all the patients showed gradual recovery in a few weeks. Acute onset of cerebellar lesion, self limiting course and cerebrospinal fluid pleocytosis suggest par- or post-infectious demyelinating pathology in these patients, who were not related to each other.

Intracranial actinomycoma with osteomyelitis simulating meningioma. Case report.

A rare case of actinomycotic osteomyelitis with an intracranial granuloma is presented. The computerized tomography findings are probably the first of their kind. Both the site of osteomyelitis and clinical presentation are unusual. The pertinent literature is reviewed.

Subdural hematoma in an adult following a blast injury. Case report.

A case is reported of chronic subdural hematoma in a civilian who was injured when a pipe exploded while being cleaned by air blasting. The literature regarding the pathogenesis of blast injury is reviewed.

Effect of dietary cadmium intake on serum thyroxine and triiodothyronine concentrations in rhesus monkeys.

Circulating thyroxine (T4) and triiodothyronine (T3) concentrations in rhesus monkeys were determined after various periods of cadmium administration in diet. No significant change in serum T4 and T3 levels was observed after 2 months of cadmium treatment. However, cadmium treatment for 4 months and 6 months showed a significant decrease in serum T4 and T3 levels indicating the adverse effect of long-term cadmium intake which increased gradually with duration of treatment.

Uncommon neurological presentations of tuberculoma.

The neurological manifestations and autopsy findings of 3 cases of tuberculoma have been described. A cerebello-pontine angle tuberculoma presented with a long history of trigeminal neuralgia. Intramedallary tuberculoma of the spinal cord presented with transverse myelitis, history of one complete remission and a normal myelogram. A tuberculoma of the medulla led to, apart from other features, severe orthostatic hypotension. Tuberculous meningitis, as a terminal process, developed in the first two cases and the third patient died from a vasomotor collapse on suddenly standing up from bed.

Meige's syndrome: clinical, pharmacological and radiological observations.

The clinical pharmacological, and neuroradiological observations in six patients with spontaneous blepharospasm-oromandibular dystonia (Meige's) syndrome are recorded. This group consisted of five males and one female, mean age at onset being 50.3 years. The duration of symptoms ranged from three months to 12 years, three patients having had symptoms for over four years. The dyskinesia was arrhythmic and asymmetrical in the orbicularis oculi and masseter muscles electrophysiologically. Pharmacological studies evinced no consistent response to parenteral physostigmine, no response to oral levodopa and no significant improvement in the dyskinesia following oral haloperidol. Lumbar air encephalogram was done in five patients, and showed frontal cortical atrophy without ventricular dilation in three. It is concluded that Meige's syndrome is a distinct nosological entity, and that physostigmine test is unlikely to be helpful in the differential diagnosis from neuroleptic-induced tardive dyskinesia. Neurotransmitter imbalance in the basal ganglia in this disorder remains to be established, and at present there is no satisfactory drug treatment for this progressively disabling movement disorder.

Migraine with interparoxysmal sharp complexes in the EEG: clinical features and response to anticonvulsant drugs.

The clinical profile and therapeutic response of 25 patients with migrainous headaches with interparoxysmal electroencephalographic (EEG) abnormalities in the form of sharp complexes are discussed. The clinical features of these patients consisted of male preponderance, early age of onset in males, long history of illness in females, very low incidence of aura and family history of migraine, frequent severe paroxysms, poor response to routine anti-migraine drugs and good response to anti-convulsant drugs. It is speculated that the EEG abnormalities in migraine may have a congenital basis which finds expression in vasomotor instability through hypothalamus and autonomic nervous system.

Balint's syndrome following eclampsia.

A young female patient sustained bilateral parieto-occipital infarcts and presented with Balint's syndrome following treatment of eclampsia and caesarean section. Altered cerebral blood flow autoregulation and raised intracranial pressure due to eclampsia probably resulted in impaired cerebral perfusion and borderzone cerebral ischaemia in this patient. Careful reduction of blood pressure in patients with eclampsia is emphasized.

Wasted leg syndrome: a clinical, electrophysiological and histopathological study.

Forty cases of 'wasted leg syndrome' were studied clinically and electrophysiologically. Muscle biopsy was examined in nine cases, majority of patients were adults engaged in heavy manual work. The illness was noticed incidently with a strictly unilateral wasting of the whole lower limb (in 65% of cases), of all muscles below the knee (in 22.5% of cases) or of quadriceps muscles only (in 12.5% of cases). The nerve conduction studies and the electromyographic pattern suggested anterior horn cell disorder. Neurogenic atrophy was seen in 7 out of 9 muscle biopsies. A follow up in 12 patients (2-6 years) revealed no progression of the disease. It is suggested that possibly these cases represent an entity, clinically different from other anterior horn cell disorders.

Acro-osteolysis (report of two cases and brief review of literature.

Two siblings suffering from acro-osteolysis, a rare disorder, are discussed. Although three distinct types of acro-osteolysis, namely familial, idiopathic, and due to vinyl-chloride, are clearly defined, these patients did not fit into any category. They had intact nails, preserved tactile and sensory system, plantar ulceration and so sensory deficit. Nerve conduction studies showed prolongation of motor distal latencies in median, ulnar, posterior tibial and lateral popliteal nerves. Nerve biopsies from both patients showed severe loss of myelinated fibres of all sizes, but more so of small ones.