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The infant with acute, unexplained, excessive crying.

This study describes 56 infants who presented to the Emergency Department of The Children's Hospital of Denver during a 1-year period with an episode of excessive, prolonged crying, without fever and without a cause that was apparent to the parents. The final diagnoses included a broad array of conditions, of which 61% were considered serious. The history provided clues to the final diagnosis in 20% of cases. Physical examination revealed the final diagnosis in 41% and provided clues to the diagnosis in another 13%. Accurate diagnosis requires a thorough physical examination, which should include careful skin inspection underneath all clothing, palpation of all large bones, fluorescein staining of the cornea, eversion of eyelids, rectal examination, retinal examination, and thorough neurologic examination. "Screening" laboratory tests, except for urinalysis and urine culture, were of little help. This study indicates that for those patients in whom the physical examination is not diagnostic, the persistence of excessive crying after the initial examination predicts the presence of a serious cause. Those infants who cease crying before or during the initial assessment are unlikely to have a serious cause. Recommendations for a stepwise assessment are offered.

Child, Preschool↗

[Evaluation of the nervous system in workers of a enolofos production department in chemical factories].

Neurological examination including EEG, ENG and measurement of nerve motor conduction velocity were performed in 41 male workers, exposed to enolofos. 43% of the population examined developed subjective neurological symptoms 34%--discrete lesions of CNS and in 31% polineuropathy was observed. Motor nerve conduction velocity was decreased in 10 workers (24%) in peroneus nerves and in 1 worker in ulnaris nerve. Comparison of EEG and ENG with the control group was not statistically significant.

Adult↗

[A case of post-traumatic medial longitudinal fasciculus syndrome].

Medial longitudinal fasciculus (MLF) syndrome recognized 2 days after a head injury is described. The patient was a 48-year-old man who had fallen from a ladder about 3m high. On his admission, scalp contusion on the left occipital area was noticed. Neurological examination revealed no neurological abnormalities except slightly disturbed consciousness. Plain skull X-ray films demonstrated a lineal skull fracture of the left occipital bone. Computed tomographic (CT) scans showed a slight subarachnoid hemorrhage within the bilateral sylvian fissures, but no parenchymal contusion in the brain stem was observed. On the 2nd day, when the patient regained full consciousness, impairment of adduction of the right eye and a fine nystagmus of the left eye on left lateral gaze were recognized. Convergence was intact. Right side MLF syndrome was diagnosed. This syndrome gradually disappeared followed by the initial improvement of adduction of the right eye, and the patient had completely recovered about 20 days after the head injury. Three major mechanisms leading to MLF syndrome caused by head injury are reported in the literature. They are: (1) primary brain stem injury, (2) secondary brainstem injury by trans-tentorial herniation, and (3) circulatory disturbance of perforating branches of the vertebro-basilar artery due to shearing force. In our case, the slightly disturbed consciousness at the time of the head injury indicates that this syndrome was not brought on by primary or secondary brain stem injury.(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Injuries↗

Activity of intrathecal 4-hydroperoxycyclophosphamide in a nude rat model of human neoplastic meningitis.

Neoplastic meningitis can result from leptomeningeal dissemination of a variety of cancers. We now report the development of animal models of human neoplastic meningitis and activity of intrathecal 4-hydroperoxycyclophosphamide (4-HC) against the human rhabdomyosarcoma cell line TE-671 and the human glioma cell line D-54 MG grown in the subarachnoid space of athymic rats. The injection of 5 x 10(5) TE-671 or D-54 MG cells resulted in leptomeningeal tumor growth from the base of the brain to the cauda equina. Daily weights and neurological examinations revealed progressive neurological deficits and weight loss, with death occurring between Days 21 and 27 for TE-671 and Days 14 and 26 for D-54 MG. 4-HC toxicity in non-tumor-bearing rats was assessed at dose levels of 2.0, 10.0, 15.0, and 20.0 mM, with clinical and histological evidence of neurotoxicity observed at the 2 highest dose levels. Intrathecal treatment with 4-HC on Day 8 following injection of TE-671 resulted in an increase in median survival of 20% (P = 0.04) at 1.0 mM 4-HC and 41% (P less than 0.001) at 2.5 mM 4-HC. Intrathecal treatment with 4-HC (2.5 mM) on Day 5 following injection of D-54 MG resulted in an increase in median survival of 23% (P = 0.009). These studies show the usefulness of the athymic rat model of human neoplastic meningitis and demonstrate the efficacy in vivo of intrathecally administered 4-HC against a human glioma and a human rhabdomyosarcoma cell line and the lack of toxicity at therapeutic levels of 4-HC in normal athymic rats.

Animals↗

[A case of stimulus-sensitive segmental spinal myoclonus].

A 37-year-old-man was admitted to our hospital because of periodic contraction of the right shoulder muscles of approximately one month's duration. He denied any significant history such as trauma or fever. General physical examination was unremarkable. Neurological examination was also unremarkable except for the periodic synchronous myoclonus involving the right sternocleidomastoid, trapezius, deltoid, rhomboid and serratus anterior muscles. X-ray fluoroscopy demonstrated a synchronized rhythmic contraction of the right diaphragm. The myoclonus was considered to be of spinal origin, because those muscles were innervated by the right fourth and fifth cervical spinal segments. Its frequency was approximately 1.2 Hz. The myoclonus disappeared during sleep. It was enhanced by a voluntary contraction of the corresponding muscles, but mental stress such as arithmetic induced no change in the myoclonus. CBC, serum laboratory data including viral antibody titer, and cerebrospinal fluid were all normal. Radiological examination including MRI showed no abnormal finding. EEG as well as somatosensory evoked potentials following median nerve stimulation were normal. Jerk-locked averaging triggered by the myoclonus of the right deltoid muscle showed no pre-myoclonus spike at the scalp electrodes. Clonazepam (1.5-6 mg/day) and phenytoin (200 mg/day) reduced the rhythmicity as well as frequency of the myoclonus, which then became stimulus-sensitive. The reflex myoclonus was induced by tapping anywhere on the body or by electric shock, but not by flash or sound. There was a refractory period of approximately 800 msec. Lack of the stimulus-sensitivity early in the clinical course could be due to this refractory period.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Visualization of epileptic lesions using single photon emission computed tomography (SPECT) with N-isopropyl-p-(123I) iodoamphetamine after intravenous loading of bemegride--report of a case].

Single photon emission computed tomography (SPECT) was performed 30 minutes and 4 hours after injection of 111 MBq (3 mCi) N-isopropyl-p-(123I) iodoamphetamine (IMP) which was injected 5 minutes after the completion of intravenous bemegride loading. A 31-year-old female with simple partial seizures evolving to complex partial seizures evolving to generalized tonic-clonic convulsive seizures with a history of hospitalization, suffering from low-grade fever, generalized convulsive seizures, and impaired consciousness at the age of 27 years was studied. Angiographic examinations, X-CT, MRI, CSF examinations, and interictal neurological examinations were normal. She suffered from clonic convulsions in her right shoulder and arm, and hallucinations and dysmnesia which were characteristic of temporal lobe epilepsy. More recently she suffered from impaired consciousness once or twice a month. While the controlled IMP-SPECT study was normal, the IMP-SPECT study examined after the activation by intravenous bemegride loading showed the pattern of a regional increased uptake of IMP in the epileptic lesions corresponding with the epileptic symptoms, and a surrounding border of decreased uptake which might be the inhibition of surrounding neuronal activity and metabolism. In conclusion, the bemegride loading IMP-SPECT study could be a potential diagnostic method in patients with seizures whose epileptic lesions were not determined by the conventional methods or in whom the surgical interventions were considered, by reason of its capability to demonstrate positively the epileptic lesions.

Adult↗

Habitual toe-walking: evaluation and approach to treatment.

Habitual toe-walking has been presented as a prolongation of a normal stage of development that requires conservative treatment to prevent or ameliorate associated gait abnormalities such as tripping and falling. An approach to the evaluation of a child with toe-walking should include (1) medical history (prenatal, intrapartum, and postnatal), (2) gait evaluation, (3) musculoskeletal examination, and (4) neurologic examination. Pathologic entities producing toe-walking have been explored in order to differentiate those conditions from idiopathic (habitual) toe-walking. The most common etiologies of toe-walking (nonhabitual) would include gastrosoleus equinus, clubfoot, or cerebral palsy. Treatment of habitual toe-walkers might include shoe therapy, orthosis therapy, auditory feedback, and surgery.

Central Nervous System Diseases↗

[Associative visual agnosia--a case report].

A 61 year-old right-handed man, a president of a company fell downstairs and was found unconscious on January 1, 1985. He was admitted to a hospital and he had been unresponsive for 10 days. As he recovered from the consciousness disturbance, he complained of difficulties in discrimination of common objects and in recognition of familiar faces. The patient was admitted to our hospital for the closer examination on July 29, 1985. There was no abnormality in the general physical examination on admission. Neurological examinations revealed no significant deficits except visual field disturbance. In the results of WAIS, the verbal IQ was 108, but the performance IQ was unmeasurable because of a presence of object agnosia. His spontaneous speech was fluent without paraphasia, perseveration or dysprosody. His auditory comprehension of spoken language and repetition were spared. Neuropsychological examinations revealed an impairment in the recent verbal memory, alexia, agraphia, object agnosia, color naming difficulty, prosopagnosia and visuospatial constructional disability. The patient exhibited three major neuropsychological characteristics. Firstly, the recognition of the objects which were presented by the photograph or line drawing was more severely impaired than the real objects. Secondly, the object naming difficulty was more pronounced in the presentation at an unusual angle than in the free presentation. Thirdly, the patient made poor performance in reproduction of the models by photographic presentation as compared with the presentation of the real models in the three-dimensional block construction test. The CT scan performed on January 4, 1985 demonstrated subcortical hematomas with surrounding edema in the temporo-occipital regions of both hemispheres.(ABSTRACT TRUNCATED AT 250 WORDS)

Agnosia↗

[A case of spontaneous carotid-cavernous fistula disappearing following irradiation].

A case of spontaneous carotid-cavernous fistula, which completely disappeared following irradiation (45 Gy), is reported. A 59-year-old woman was admitted on October 10, 1983, with a history of doplopia and recurrent retrobulbar and occipital pain. On admission, neurological examination showed no neurological deficit, and right cerebral angiography demonstrated a dural internal carotid-cavernous fistula fed by the meningohypophyseal trunk and drained into the inferior petrosal sinus. In spite of the conservative management, right oculomotor palsy and hypesthesia corresponding to the division of ophthalmic branch of the right trigeminal nerve was developed. On November 22, liniac irradiation to the region of fistula (30 days, total dose, 45 Gy) was started. Symptoms and signs gradually improved 3 weeks after irradiation and completely disappeared 3 months after irradiation. Carotid-cavernous fistula was no longer demonstrated by the cerebral angiography 7 months after irradiation. Two years after onset, no recurrence of symptoms was observed.

Arteriovenous Fistula↗

Prolonged exposure to trimethylphosphate induces sensory motor neuropathy in the dog.

Five beagle dogs were treated daily with 1 ml of trimethylphosphate (TMP) for 1 to 4 months. Neurotoxicity was evaluated by observation of behaviour, neurologic examination, neurophysiologic tests and by neuropathology. Neurotoxicity was first detected at the neurologic examination following four weeks of treatment: the most predictive indicators of neuropathy were the impairment of hopping, of tactile placing and the atactic gait. Electrophysiologic tests indicated a prolonged latency of neuromuscular impulse transmission after week nine, followed by a decrease of maximum conduction velocity of sensory fibers. At this time peripheral nerve fibers had abnormalities including paranodal and internodal swelling, paranodal demyelination and distal fragmentation. An additional dog treated with daily doses of 2 ml developed a severe distally accentuated degeneration of long spinal tracts and peripheral nerve fibers, manifested by impairment of sensory and central motor impulse conduction. Organophosphate neuropathy can thus be assessed in the dog by functional and morphological changes after prolonged exposure, in particular at doses causing severe morbidity.

Animals↗

Genuine stress urinary incontinence with low urethral pressure. Five-year follow-up after the Ball-Burch procedure.

OBJECTIVE: To evaluate long-term results of the Ball-Burch procedure in women with genuine stress incontinence (GSI), low urethral pressure type. STUDY DESIGN: Sixty women with a clinical and urodynamic diagnosis of GSI, low urethral pressure type, underwent the Ball-Burch procedure between May 1987 and February 1988. They were evaluated preoperatively and one and five years after surgery. The urogynecologic workup consisted of a history, physical examination, urine culture, neurologic examination, urethrocystoscopy, cotton swab test, urethrocystometry, urethral profilometry and uroflowmetry. Forty-eight women were available at the one-year follow-up, and 32 of the 48 (66.7%) were available after five years; the mean age at the time of surgery was 58.7 years and mean parity, 2. Twenty-seven women were premenopausal and 33 postmenopausal; at the five-year follow-up all but 2 were postmenopausal. All postmenopausal women received estrogen replacement therapy prior to urodynamic evaluation. Cure was defined as absence of loss of urine by history (subjective) and during urodynamic evaluation (objective). The t test and paired t test were used for statistical analysis. RESULTS: Forty-two of 48 women (87.5%) were cured after one year, and 27 of 32 (84%) were cured after five. There was a statistically significant difference between the preoperative and five-year follow-up values for the swab test, maximum urethral closure pressure and pressure transmission ratio at the proximal and mid urethra. Twenty-six of 29 patients with a negative swab test at the one-year follow-up were cured after five years, resulting in a predictive value of 89% for cure after a negative test. CONCLUSION: In our series the Ball-Burch procedure was an effective surgical treatment for patients with GSI and low urethral pressure. The swab test, when negative one year after surgery, was predictive of a successful long-term outcome.

Adult↗

[A 85-year-old right-handed woman with aphasia and left hemiparesis].

We report a 85-year-old woman who developed speech disturbance and left hemiparesis. She had a gradual onset of gait disturbance 3 years prior to the present admission. Five days before admission, she started to pace up and down in her house; she did not want to take food on the following day, and she developed fever of 39 degrees C; it was also noted that she became mute. On the next day, she developed left hemiparesis; she was still mute but was able to communicate by hand writing to some extent. She was admitted to our service on February 24, 1992. On admission, she was alert but mute; her body temperature was 37.1 degrees C, and her BP 110/70 mmHg. The lungs were clear and general physical examination was unremarkable. Neurologic examination revealed that she did not utter even a word. She was unable to understand examiner's simple questions; communication by hand writing was also difficult, but she could draw her name and a circle; repetition was also impaired. Examination of other higher cerebral functions such as praxis and gnosis was impossible. Her optic fundi were unremarkable; no anisocoria was noted; extraocular muscles appeared intact, and the vestibulo-ocular reflex was normally elicited. The nasolabial fold was shallower on the left; the tongue showed a slight deviation to the left. She had near complete spastic left hemiplegia; deep tendon reflexes were increased bilaterally.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗

Clinical manifestations of benign prostatic hyperplasia.

The clinical diagnosis of BPH is usually established after completing the initial evaluation of patients who present with symptoms of prostatism. The steps recommended in the initial evaluation include a medical history, symptom assessment using a Symptom Score Index, a digital rectal examination, a focused neurological examination, urinalysis, measurement of serum creatinine, and an optional PSA measurement. Further examinations such as uroflowmetry and cystoscopy are indicated only if the diagnosis is uncertain, or to determine the feasibility of specific invasive therapies.

Diagnosis, Differential↗

[A 56-year-old woman with parkinsonism and dementia with the age of onset at 41 years].

We report a 56-year-old woman with parkinsonism and dementia who died of respiratory failure. The patient was well until the age of 41 when she noted insidious onset of difficulty in moving around. Soon after, she noted tremor in both her hands and gait disturbance. She received stereotaxic right thalamotomy when she was 46-year-old; after thalamotomy, improvement was noted in her tremor, rigidity, and in gait. However, a few months later, she started to experience motor fluctuations with worsening of her symptoms in the afternoon. This worsening was temporarily relieved by increasing her levodopa/benserazide dose. She started to show visual hallucination and agitation when she was 54-year-old. Her symptoms had progressively become worse with marked motor fluctuations and she was admitted to our hospital when she was 56-year-old. On admission, she was alert and general physical examination was unremarkable. Neurologic examination revealed that she was disoriented to time and place; memory was markedly disturbed and calculation was poor. Hasegawa dementia scale was 7/30. Higher cerebral functions appeared intact. She showed masked face, small voice, and some dysphagia. Other cranial nerves were intact including ocular movements. She was unable to walk by herself; when supported she walked in small steps with marked disturbance in the righting reflex. Mixed rigidity and Gegenhalten was noted in her four limbs and in the neck. Tremor was absent. She showed marked akinesia.(ABSTRACT TRUNCATED AT 250 WORDS)

Age of Onset↗

[A 64-year-old man with recurrent blurred vision and an abdominal mass].

We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)

Adenocarcinoma, Mucinous↗