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PubMed2001

Early recovery after closed traumatic head injury: somatosensory evoked potentials and clinical findings.

OBJECTIVE: To determine the ability of somatosensory evoked potentials (SEP) compared with clinical findings to monitor and predict recovery in patients suffering from closed head injury with predominantly diffuse axonal injury (DAI). DESIGN: Prospective cohort study. SETTING: Neurologic intensive care unit (ICU) of a university hospital. PATIENTS: Serial SEP recordings were obtained from 31 consecutive patients with closed head injury. The first SEP was recorded within 48 hrs after trauma, followed by recordings after another 2 days, after which the time interval for each consecutive recording was doubled. Clinical examinations were performed every 6 hrs during the ICU stay and daily after transfer to a general neurologic ward. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Twenty-three of 31 patients demonstrated pathologic SEP findings at initial examination. Of these patients, 11 recovered clinically, two remained vegetative, and ten died. In all 11 patients with clinical recovery, SEP also recovered. In 8 of 31 patients, initial SEPs were normal and remained normal until discharge, all eight had a good outcome. Initial SEP findings were related with outcome at 6 months (p = .02), and follow-up studies increased the predictive value of SEP studies (p = .009). Other factors related to outcome included age, severity of DAI, and length of ICU/hospital stay. In the 11 patients with SEP and clinical recovery, early (day 2) and late (>or=2 months) recovery was documented. Early and reliable SEP indicators of improvement included N20-P25-Amplitudes (mean recovery, 8.5 days) and central conduction time (9.6 days). Pupillary light reaction (6.4 days), Babinski reflex (12.4 days), and Glasgow Coma Score (9.6 days) were the most valuable clinical findings. Recovery of the Glasgow Coma Score frequently coincided with reduction of sedatives. In most patients, recovery was detected with SEP before clinical recovery (7/11 patients, time difference 1 wk). CONCLUSIONS: Initial SEP findings correlate with long-term outcome in patients with closed head injury with DAI. Initial bilaterally absent cortical responses in the SEP reliably predicted death, whereas completely normal SEP findings predicted good long-term outcome. Early recovery after DAI can be detected with serial SEP recordings despite sedative medications. Electrophysiologic recovery frequently precedes clinical recovery.

Adult↗
PubMed2001

Magnetic resonance imaging detection of a lesion compatible with central pontine myelinolysis in a pregnant patient with recurrent vomiting and confusion.

The authors report a patient who presented with unexplained confusion. She was 15 weeks pregnant and had been having recurrent vomiting for several weeks. This was felt to possibly represent hyperemesis gravidarum, but she had several other possible contributing factors. Her serum sodium was 146 mmol/L, and her potassium was 2.6 mmol/L. She was alert but had disorientation, visual hallucinations, memory impairment, and confabulation despite being a college graduate with no history of illicit drug use or excessive alcohol consumption. Her initial magnetic resonance imaging (MRI) brain scan was interpreted as being normal. However, her follow-up MRI brain scan revealed typical findings of central pontine myelinolysis, which correlated with hyperreflexia and positive Babinski reflexes. This patient illustrates the constellation of signs and symptoms that can be seen with a demyelinating lesion of the pons. In addition, our case illustrates how this MRI scan finding can be quite nonspecific but may help to explain the clinical findings.

Adult↗
PubMed1978

Evidence for changes in segmental motoneurone pools by chronic cerebellar stimulation and its clinical significance.

Electrophysiological studies in eight patients with severe motor system abnormalities are described both before and after the start of chronic electrical stimulation of the anterior cerebellum. With stimulation, at least one of following changes was found in seven of these eight patients: reversal of abdominal F amplitude ratios (flexor/extensor muscles), decreased prominence of F responses, and loss of H reflexes in at least one muscle other than the soleus. These changes indicate that cerebellar stimulation can affect the excitability of segmental motoneurone pools. Although clinical changes, particularly decrease in tone, were observed in all the patients with stimulation, the complexity of trying to relate the physiological findings to functional improvement is emphasised.

Action Potentials↗
PubMed1984

Delayed neuropathy after organophosphorus insecticide (Dipterex) poisoning: a clinical, electrophysiological and nerve biopsy study.

Clinical, electrophysiological and histological findings in four patients accidentally poisoned with the organophosphorus insecticide Dipterex are reported. Three to five weeks after insecticide ingestion signs of a distal sensorimotor (preponderantly motor) neuropathy occurred. The patients complained of paraesthesia in the lower limbs, and two of them of very disagreeable pricking sensation in the soles of the feet, responsive to carbamazepine. They showed distal weakness mainly of the legs, footdrop , difficult gait and muscle hypotonia. Ankle jerk was abolished while other tendon reflexes persisted. Two months or even later after poisoning, knee jerks in all the patients were very brisk and more and less accompanied by other pyramidal signs (patellar clonus, abolishment of abdominal cutaneous reflexes, Babinski's sign). Clinical, electrophysiological and nerve biopsy data revealed a "dying-back" neuropathy in our patients. Distal muscle fatigue was confirmed by failure of neuromuscular transmission on repetitive nerve stimulation.

Adult↗
PubMed2004

Objective tests for upper motor neuron involvement in amyotrophic lateral sclerosis (ALS).

OBJECTIVE: To develop objective markers for upper motor neuron (UMN) involvement in ALS, the value of single-voxel MR spectroscopy (MRS) and transcranial magnetic stimulation (TMS) was studied. METHODS: Test results of 164 ALS patients who had MRS only (n = 91), TMS only (n = 13), or both (n = 60) were analyzed; also, 11 autopsy examinations were evaluated. RESULTS: Abnormal test results consistent with UMN involvement were found in 134 patients with clinical UMN signs: 86% on MRS, 77% on TMS, and 70% on MRS and TMS together. Among 30 patients with solely LMN signs (progressive muscular atrophy), UMN results were found in 63% on MRS, 63% on TMS, and 46% on both tests together. There was a significant association of the degree of abnormal N-acetyl aspartate/creatine ratios with UMN signs (p = 0.01). The sensitivity to detect UMN involvement was 0.86 for MRS (specificity 0.37) and 0.77 for TMS (specificity 0.38). At autopsy, all 11 patients had pathologic UMN abnormalities, including 4 with normal MRS and 1 with normal TMS in life. CONCLUSIONS: MRS is highly sensitive, somewhat more than TMS, and shows good correlation with clinical UMN signs. Combining MRS and TMS results in the same patient with further refinement may help in the early diagnosis of ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1997

Hyperactive pectoralis reflex as an indicator of upper cervical spinal cord compression. Report of 15 cases.

Myelopathy from cervical spondylosis is often accompanied by hyperreflexia of the upper-extremity deep tendon reflexes (DTRs). Reflexes such as the pectoralis jerk and the deltoid jerk may only be apparent in the context of hyperreflexia. Although the nerve roots involved in the reflex arcs are well described, levels of cervical spinal cord compression that lead to the hyperreflexia are not as clear. This is of particular significance for patients with multilevel cervical spondylosis in determining the levels responsible for their symptoms. The authors examined 15 consecutive patients who presented for treatment of cervical myelopathy. The clinical examination was then correlated with levels of cervical spinal cord compression by cervical magnetic resonance imaging or computerized tomography with intrathecal contrast enhancement. The presence of a prominent pectoralis jerk was seen only in patients with spinal cord compression at the C2-3 and/or C3-4 levels (nine patients). No patient with compression at or below the C4-5 disc space without coexisting compression at a higher level had hyperactive pectoralis reflexes. This association between the C3-4 level and a hyperactive pectoralis reflex was significant (p < 0.004, Fisher's exact test). The deltoid reflex was tested in the last nine consecutive patients. It was present in patients with compression of the upper spinal cord at levels C3-4 and C4-5 (four of five patients) but appeared in only one of four patients with compression below C4-5. This association did not attain statistical significance. The presence of a hyperactive pectoralis reflex is specific for lesions of the upper cervical spinal cord. Examination of upper-extremity DTRs may be helpful in planning the appropriate levels for surgical decompression in patients with multilevel spondylosis and myelopathy.

Humans↗
PubMed2002

Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult. Case report.

Diastematomyelia is a rare entity in which some portion of the spinal cord is split into two by a midline septum. Most cases occur in childhood, but some develop in adulthood. A variety of concurrent spinal anomalies may be found in patients with diastematomyelia. The authors describe a 38-year-old right-handed woman who presented with a 7-month history of lower-extremity pain and weakness on the right side. She denied recent trauma or illness. Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. Neuroimaging revealed diastematomyelia extending from T-1 to T-3, an expanded right hemicord from T-2 to T-4, and a C6-7 syrinx. The patient underwent T1-3 total laminectomies, resection of the septum, untethering of the cord, and excision of the hemicord lesion. The hemicord mass was determined to be an intramedullary epidermoid cyst; on microscopic evaluation the diastematomyelia cleft was shown to contain fibroadipose connective tissue with nerve twigs and ganglion cells. Postoperatively, the right lower-extremity pain, weakness, and sensory deficits improved. Diastematomyelia can present after a long, relatively asymptomatic period and should be kept in the differential diagnosis for radiculopathy, myelopathy, tethered cord syndrome, or cauda equina syndrome. Numerous spinal lesions can be found in conjunction with diastematomyelia. To the authors' knowledge, this is the first case in which a thoracic epidermoid cyst and cervical syrinx occurred concurrently with an upper thoracic diastematomyelia. Thorough neuraxis radiographic evaluation and surgical treatment are usually indicated.

Adult↗
PubMedDate not supplied

[Eclampsia with a transient neurological deficit].

The results of certain clinical studies indicated the possibility of the onset and development of cerebrovascular diseases during pregnancy. Pathogenesis of this cascade of developments is frequently unknown. Eclampsia is the leading clinical syndrome, associated with numerous neurological complications. This is a case report of a 24 years old patient, who in a short period became extremely psychomotorically, agitated, with bilateral iridoplegia and positive Babinsky reflex during the development of eclampsia. Soon, a series of grand-mal epileptic attacks developed in the patient. However, the revealed clinical phenomenology was completely withdrawn within 6 hours, after the adequate estimation of clinical status and timely therapeutic measures. We have concluded that the patient had transient global cerebral ischemia, as a part of acute hypertensive endephalopathy.

Adult↗
PubMed2000

[A patient with motor neuron syndrome clinically similar to amyotrophic lateral sclerosis, presenting spontaneous recovery].

We report a patient with motor neuron syndrome similar to amyotrophic lateral sclerosis (ALS) and with spontaneous recovery. At the age 40, the woman developed progressive muscular weakness, atrophy and fasciculation in extremities. She also noted a dyspnea, tongue atrophy and dysphagia. A neurological examination 6 months after onset revealed i) a tongue atrophy and fasciculation, ii) diffuse muscule weakness and atrophy in face, neck and extremities, and iii) marked hyperreflexia in the four limbs and bilateral Babinski reflex, but iv) neither sensory disturbance nor ophthalmoplegia. Electromyogram (EMG) detected such denervation potentials as fibrillation potentials, fasciculation potentials, positive sharp waves and polyphasic or giant MUPs diffusely in the limb muscles. Peripheral nerve conduction study detected neither conduction block nor delay. Thus, she was diagnosed as suffering from ALS. However, since approximate 1 year after onset, her muscle weakness has gradually been getting better. Simultaneously, the dyspnea and dysphagia gradually improved. Two years after onset, an EMG examination detected chronic denervation potentials in the left musculus sternocleidomastoideus and a few on-going denervation potentials in the left musculus extensor carpi radialis, but no denervation potentials in other limb muscles. Fasciculation potentials were found in tongue muscles. Thus, the present case was thought to have a reversible motor neuron syndrome clinically quite similar to ALS. A mild increase in IgE (346 U/ml) and a low-titer IgM-class anti-GM1 antibody were found in her serum though its pathological significance was uncertain. Any immunological aberrance may account for the pathogenesis. It should be noted that clinically diagnosed cases of ALS may rarely recover spontaneously.

Adult↗
PubMed2001

[A case of Machado-Joseph disease presenting pure cerebellar ataxia].

We report a 61-year-old woman with Machado-Joseph disease (MJD) presenting with pure cerebellar ataxia. The patient exhibited an unsteady gait at the age of 51 years. She was admitted to our hospital at the age of 61 years. Her older brother had been diagnosed as having spinocerebellar degeneration (SCD). Our patient showed gaze-evoked nystagmus, wide-based gait, slight lack of coordination of the four extremities, mildly ataxic speech and slight decrease in the bilateral Achilles tendon reflexes. Babinski's sign was absent. Sensory impairments were not present and muscle tone and muscle strength were normal. There was no autonomic dysfunctions. MRI revealed moderate atrophy of the cerebellum and pons. We performed gene analysis of SCD using white blood cells from the patient, and the analysis showed 70 CAG repeats in the MJD1 gene, which is an abnormally high number of repeats. Compared with three reported cases of MJD presenting pure cerebellar ataxia, only our patient showed a nasal voice. The number of CAG repeats in the MJD1 gene of our patients was the most prolonged of the four cases. MJD should be considered in patients with familial SCD even if their neurological signs and symptoms outside the cerebellum are not obvious.

Ataxin-3↗
PubMedDate not supplied

[Generalized microsporidiosis caused by Encephalitozoon sp. in a pediatric patient with Bruton's disease].

We present the case of a four-year-old boy with a history of repeated upper respiratory tract infections and pyoderma. He presented fever, seizures, inability to talk, loss of swallowing, fine tremor in the upper extremities; positive bilateral Babinski reflex and quadriparesis. The diagnosis of Bruton's disease and generalized microporidiosis was based on immunologic analysis, smear tests with chromotrope R2 stain and indirect immunofluorescense with monoclonal 3B6 antibody for Encephalitozoon species in samples of spinal fluid, bronchial and paranasal sinus aspirates and stool, which were all positive. The patient was treated with albendazol during 72 days; he left the hospital in a good condition, walking, talking and able to swallow. His laboratory test controls were negative; he is followed up in the outpatient department.

Agammaglobulinemia↗
PubMed2002

[Acute promyelocytic leukemia accompanied by retinoic acid syndrome with complications of acute myocardial infarction and cerebral infarction during treatment with all-trans retinoic acid].

A 71-year-old man visited our hospital complaining of fever and a bleeding tendency. The peripheral blood WBC count was 10,400/microliter with 90% promyelocytes. The bone marrow was hypercellular with 88% promyelocytes. Disseminated intravascular coagulation was recognized. The patient was diagnosed as having acute promyelocytic leukemia and was treated with daily oral administration of all-trans retionic acid (ATRA) (45 mg/m2/day) and cytarabine (160 mg/day, intravenous drip infusion for the initial five days). The ATRA treatment induced leukemic cells to undergo mature myeloid differentiation. On day 24 after the start of treatment, the WBC count rapidly increased and acute myocardial infarction appeared, with consciousness disturbance and bilateral Babinski reflex appearing three hours later. Magnetic resonance imaging showed a fresh lacunar infarction of the right lenticular nucleus, and serum levels of IL-6 and PAI-1 were found to be elevated at the onset of infarction. Since there was a possibility that the retinoic acid syndrome (RAS) might have helped bring about the infarctions, we stopped the ATRA treatment and started administration of methyl-prednisolone (500 mg/body/day for 3 days) and gabexate mesilate. The WBC count decreased immediately and the consciousness disturbance improved. In this case, ATRA treatment might have initiated the RAS and resulted in some endothelial damage, thus causing the infarctions.

Aged↗
PubMed1992

[Acute hepatitis A (HA) presenting findings of meningoencephalitis].

A 39-year-old man, who had high grade fever and headache for 4 days was admitted to our hospital because of generalized seizure and disturbance of consciousness. He was pyrexial, but not icteric. Neurological examination revealed disorientation, nuchal rigidity and bilateral Babinski reflexes. Laboratory test results included the following: GOT 1,740 U/l, GPT 2,800 U/l, bilirubin 1.2 mg/dl, serum IgM-HA antibody cut-off index 6.8. CSF was clear, with 10 leukocytes/mm3 and protein level of 108 mg/dl. Head CT and MRI revealed no abnormality. An EEG demonstrated diffuse slowing. During the following 2 days, he had increased obtundation and labored breathing. In the second week of hospitalization his neurological conditions and liver function test results improved. A diagnosis of HA was confirmed by a finding of serum IgM-HA antibody. The neurological findings, CSF findings and clinical course indicated acute meningoencephalitis in association with HA. To our knowledge, there have been only 4 previous case reports of meningoencephalitis associated with serologically confirmed HA infection. HA virus infection might pass unnoticed, as many cases of HA infection remain anicteric or subclinical. Therefore, HA virus should also be considered as one of the etiological agents in meningoencephalitis.

Acute Disease↗
PubMed2004

[An outbreak of encephalopathy after eating autumn mushroom (Sugihiratake; Pleurocybella porrigens) in patients with renal failure: a clinical analysis of ten cases in Yamagata, Japan].

In September and October, 2004, an outbreak of encephalopathy of unknown etiology occurred in certain areas of Japan including Yamagata, Akita, and Niigata prefectures. These patients had a history of chronic renal failure, most of them had undergone hemodialysis, and also had a history of eating Sugihiratake (Pleurocybella porrigens), an autumn mushroom without known toxicity. Since clinical details of this type of encephalopathy remain unknown, we analyzed the clinical, radiological and electroencephalographic (EEG) features of ten cases of this encephalopathy in Yamagata prefecture. The summary of the present study is as follows: 1. Ten patients had chronic renal failure, and seven underwent hemodialysis. 2. Each patient had a history of eating Sugihiratake within 2-3 weeks of the onset of neurological symptoms. 3. The onset was subacute; the initial symptoms were tremor, dysarthria, and/or weakness of the extremities, which lasted an average of 4.5 days (ranging from 2 to 11 days), followed by severe consciousness disturbance and intractable seizures, resulting in status epilepticus in 5 patients. Myoclonus was also seen in 4 patients and Babinski reflex in 3. 4. Brain CT and MRI examinations were unremarkable in the early stages of the disease. Three to eight days after onset, however, conspicuous lesions appeared in the areas of the insula and basal ganglia in 6 patients. On MRI, these brain lesions were hyperintense on T2-weighted and FLAIR images, and hypointense on T1-weighted images. 5. EEG examination was performed in 6 patients, all of whom showed abnormal EEG findings. Periodic synchronous discharge (PSD) was seen in 2 patients, spike and wave complex in one patient, and non-specific slow waves in 3. 6. Prognosis was different from case to case. Three patients died at 13, 14, and 29 days after onset. Two patients still showed persistent disturbance of consciousness one month after onset. One patient showed parkinsonism after recovering from consciousness disturbance. Four patients recovered nearly completely around one month after onset In 3 of the 4 recovered patients, renal failure was not severe and they did not need to undergo hemodialysis. This suggests that the degree of renal failure is a key for the prognosis of this type of encephalopathy. The present study suggests that this endemic disease is a newly recognized clinical entity of encephalopathy.

Aged↗
PubMed1990

[A case of embryonal carcinoma arising in the basal ganglia of the cerebrum].

The occurrence sites of intracranial primary germ cell tumors are most often the pineal and suprasellar regions. The histological type observed most frequently in these tumors is germinoma. Cases of embryonal carcinoma arising in the basal ganglia are rarely reported. To our knowledge, only 4 such cases have been previously reported in the literature. A case of an embryonal carcinoma arising in the basal ganglia is reported. A 17-year-old boy was admitted to our hospital on July 30, 1988 because of headache and vomiting, and a right hemiparesis. On admission, physical inspection showed no abnormalities and neurological examination revealed obtunded consciousness, a right central facial paresis and a right hemiparesis with Hoffman and Babinski reflexes. Noncontrast CT scan showed a large mass of low-to slightly high-density in the region of the left basal ganglia accompanied with midline shift and ventricular dilatation. Enhancement of the lesion was made by contrast CT scan. It was not homogeneous. Cerebral angiogram displayed a contralateral shift and an unrolling of the anterior cerebral artery, a lateral stretch of middle cerebral artery, a downward stretch of anterior choroidal artery and a tumor stain fed by the Heubner artery. On August 3, left frontotemporal craniotomy was performed. The tumor was totally removed in a piecemeal manner using microsurgical techniques. Histopathological diagnosis was mixed-type of germ cell tumor comprising embryonal carcinoma and teratoma. Postoperative CT scan showed complete disappearance of the tumor. A course of radiation of 4950 rads and two courses of a combination chemotherapy with cis-platinum, vinblastine and bleomycin were given within 3 months after the operation.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗