Home About us Contact
|
Home About us Contact | ||
|
|
||
Deep Tendon Reflexes (deep + tendon_reflex)
Selected AbstractsCongenital hypomyelination neuropathy in a newborn infant: unusual cause of diaphragmatic and vocal cord paralysesJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 2 2002JS Hahn We report a case of congenital hypomyelination neuropathy presenting at birth. The infant had generalized hypotonia and weakness. There was decreased respiratory effort along with a right phrenic nerve and left vocal cord paralyses. Tongue fasciculations were present. Deep tendon reflexes were absent in the upper extremities and hypoactive (1+) in the lower extremities. Magnetic resonance imaging of the head revealed no intracranial abnormalities, including normal cerebral myelination. Nerve conduction study showed absence of motor and sensory action potentials in the hands when the nerves in the upper limbs were stimulated. A motor response could be elicited only in the proximal leg muscles. Needle electromyography study was normal in the proximal limb muscles, but showed active denervation in the distal muscles of the arm and leg. These findings were thought to be consistent with a length-dependent sensorimotor peripheral polyneuropathy of axonal type with greater denervation of the distal muscles. A biopsy of the quadriceps muscle showed mild variability in fiber diameter, but no group typing or group atrophy. The muscle fibers showed no intrinsic abnormalities. Biopsy of the sural nerve showed scattered axons with very thin myelin sheaths. There was also a nearly complete loss of large diameter myelinated fibers. No onion bulb formations were noted. These findings were thought to be consistent with congenital hypomyelination neuropathy with a component of axonopathy. DNA analysis for identification of previously characterized mutations in the genes MPZ, PMP22, and EGR2 was negative. Several attempts at extubation failed and the infant became increasingly ventilator-dependent with increasing episodes of desaturation and hypercapnea. He also developed increasing weakness and decreased movement of all extremities. He underwent surgery at 2 months of age for placement of a gastrostomy tube and a tracheostomy. He was discharged from the hospital on a ventilator at 6 months of age. The infant was 13 months old at the time of submission of this report. Although he appears cognitively normal, he remains profoundly hypotonic and is on a home ventilator. There was no evidence of progressive weakness. Congenital hypomyelination neuropathy is a rare form of neonatal neuropathy that should be considered in the differential diagnosis of a newborn with profound hypotonia and weakness. It appears to be a heterogeneous disorder with some of the cases being caused by specific genetic mutations. [source] Ross syndrome, an entity included within the spectrum of partial disautonomic syndromesJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 6 2005M Ballestero-Díez ABSTRACT Ross syndrome is a degenerative peripheral nervous system disorder defined by the following triad: unilateral or bilateral segmental anhidrosis, hyporeflexia of deep tendon reflexes and Adie's tonic pupils. The most disturbing symptom is segmental compensatory hyperhidrosis. It has only occasionally been reported in the dermatological literature. We present a 35-year-old woman with chronic hepatitis C who developed the characteristic triad of Ross syndrome within 1 month. The patient was otherwise healthy except for an aneurysm of the left medium brain artery not responsible for the syndrome. [source] ANTI-SULFATIDE IgM ANTIBODIES DETECTED IN A PATIENT DIAGNOSIS OF MOTOR NEURON DISEASEJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002Article first published online: 11 MAR 200 D'Avino C., Del Corona A., Bacci A., Calabrese R., Siciliano G. Department of Neuroscience-Clinical Neurology-University of Pisa-Italy Case report. The patient, a 66-year-old man with a 5-year diagnosis of diabetes mellitus, in Sep. 2000 started complaining of language disturbances as rhinolalia. In Jan. 2001, because of generalized fatigue and difficulties in walking, he was hospitalized in Internal Medicine and a diagnosis of diabetic angiopathy and neuropathy was made. Since discharge patient clinical conditions gradually deteriorated and a neurological evaluation showed tongue atrophy, dysarthria, dysphagia, fasciculations in the four limbs, increased deep tendon reflexes with bilateral foot clonus and paraparetic spastic deambulation. He underwent spinal MRI that showed mild arthrosic abnormalities in cervical spinal cord and limb EMG that showed denervation spontaneous activity with neurogenic MUAP modifications, with normal sensory and motor conduction velocity. MEP showed bilateral pyramidal track involvement. A significantly increased anti-sulphatide IgM antibodies titer (1:32,000) in the serum was detected. The diagnosis at discharge was "probable motor neuron disease" and the patient is under riluzole therapy at the moment. Discussion. Anti-sulfatide IgM antibodies are currently associated with several subtypes of peripheral neuropathy. In most cases it is a chronic dysimmune sensory or sensorimotor neuropathy in which electrophysiological and morphological studies are usually con- sistent with a predominant demyelination frequently associated with prominent axonal loss. Although rare, an association between motor neuron disease and IgM anti-sulfatide has been described in a recent paper by Latov and coworkers that reviewed electrophysiologic, morphologic and laboratory data of 25 patients with elevated antisulfatide antibodies. It seems interesting to follow-up the clinical course of the patient, the response to therapy and its correlation to antibodies titer, while the opportunity of high dose IVIg therapy is under discussion at the moment. [source] AUTOMIC FAILURE AND NORMAL PRESSURE HYDROCEPHALUS IN A PATIENT WITH CHRONIC DEMYELINATING INFLAMMATORY NEUROPATHYJOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2002M. Laurà A 75-year-old man with HCV hepatitis developed at the age of 70 presented with rest and action tremor localized at both hands and progressive cognitive impairment with memory loss. Four years later he begun to complain of progressive fatigue, occasional falls, numbness at the extremities and orthostatic hypotension. One month after admission, he rapidly worsened with inability to walk, mainly because of autonomic failure. Neurological examination revealed gait disturbances, including a wide base of support and short stride, slurred speech, reduction of upward gaze, rest and action tremor at both hands, intrinsic hand muscle and anterior tibialis muscle wasting and weakness on both sides, absent deep tendon reflexes, loss of vibration sense at lower limbs, and bilateral pes cavus. Routine laboratory studies, autoantibodies, thyroid function, neoplastic markers and immunoelectrophoresis were normal. Cryoglobulins were absent, whereas CSF protein content was increased (142 mg/dl). Autonomic nervous system investigation detected severe orthostatic hypotension. Nerve conduction studies showed absent sensory potentials and a marked reduction of compound motor action potential amplitudes and of motor conduction velocities. A sural nerve biopsy revealed remarkable onion bulb-like changes, endoneurial and perivascular infiltrations of inflammatory cells. Psychometric tests showed mild cognitive impairment. Brain MRI was consistent with normotensive hydrocephalus. The findings indicated the presence of chronic inflammatory demyelinating polyneuropathy, autonomic nervous system involvement and normal pressure hydrocephalus. A condition of multiple system atrophy (MSA) might be taken into account, even if somatic peripheral nerve involvement may rarely occur in MSA. Moreover the normal pressure hydrocephalus could be due to the high protein content in CSF (Fukatsu R et al., 1997). [source] Peripheral neuropathy in an outpatient cohort of patients with Sjögren's syndromeMUSCLE AND NERVE, Issue 5 2006Glenn Lopate MD Abstract Peripheral neuropathy is common in patients with Sjögren's syndrome (SS), but its precise prevalence is unknown. Most prior studies were conducted at neurology or rheumatology specialty clinics and likely selected for a more severely affected population. We evaluated 22 SS patients and 10 controls for evidence of neuropathy in an outpatient setting at a regional meeting of the Sjögren's Syndrome Foundation. We performed neurological examinations and nerve conduction studies (NCSs) and measured serum antinuclear antibody (ANA) and SS-A and SS-B antibody levels. Participants filled out a questionnaire pertaining to symptoms, diagnosis, and treatment. We found that signs and symptoms related to small axons were more common in patients with SS than in controls. Complaints of painful distal paresthesias in the feet were noted in 59% of patients but in only 10% of controls, and of abnormal sweating in 41% and 0%, respectively. Examination revealed decreased pinprick sensation in 64% of patients with SS, but in only 30% of controls. Overall, 45% of the patients but none of the controls were thought to have an isolated small-fiber neuropathy. Large-fiber dysfunction (as measured by testing vibration, deep tendon reflexes, and NCSs) was similar between the two groups. We conclude that small-fiber neuropathy is common in patients with SS. Muscle Nerve 2006 [source] Determinants of prognosis of acute transverse myelitis in childrenPEDIATRICS INTERNATIONAL, Issue 5 2003Reiko Miyazawa Abstract Background: Acute transverse myelitis (ATM) is a severe disorder; recovery requires several months and often leaves neurologic residua. To determine what features of patients with acute transverse myelitis significantly influence prognosis, the authors reviewed reports of ATM in Japanese children published in the last 15 years (from 1987 to 2001). Methods: The authors studied reports of 50 Japanese patients (17 boys, 26 girls, 7 children of unspecified sex; mean age ± SD, 8.0 ± 3.8 years). Acute-phase and demographic features including age, increased deep tendon reflexes, Babinski reflex, sex, preceding infection, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone and/or high-dose methylprednisolone, and the day of illness when treatment was started were used as independent variables in a regression analysis. The dependent variable was long-term persistence of neurologic deficits. Results: Younger patients and those without increased deep tendon reflexes or a Babinski reflex were more likely to have residual neurologic deficits such as paraplegia or tetraplegia, sensory loss and sphincter disturbance. No relationship was seen between prognosis and sex, preceding infections, decreased deep tendon reflexes, time course of peak neurologic impairment, treatment with prednisolone or high-dose methylprednisolone, or timing of treatment initiation. Conclusions: Age at onset and neurologic features were important for outcome prediction in ATM. Steroid therapy did not associate with better outcome. [source] Clinical and electromyographic deep tendon reflexes in polyneuropathy: diagnostic value and prevalence,ACTA NEUROLOGICA SCANDINAVICA, Issue 4 2009K. R. Sharma Background,,, Evidence is accumulating that patients with polyneuropathy may present with normal clinical deep tendon reflexes (C-DTR). There are few studies that assessed the diagnostic utility of electromyographically recorded DTR (Er-DTR) in patients with polyneuropathy. Objectives,,, The objectives of this study were twofold: (i) to evaluate the prevalence of preserved C-DTR in polyneuropathy; (ii) diagnostic value of Er-DTR latency measurement in patients with polyneuropathy. Methods,,, We prospectively studied 38 controls and 185 patients with polyneuropathy. All subjects had evaluation of C-DTR, Er-DTR obtained from right biceps brachii (BR), right patellar (PR) and bilateral ankle reflexes (AR). Results,,, Of these 185 patients, 118 (63.8%) had chronic axonal neuropathy (CAN), 49 (26.5%) demyelinating polyradiculoneuropathy (DPN) and 18 (9.7%) small fiber neuropathy (SFN). The C-DTR were normal in 65 patients whereas 39 of these 65 (60%) patients had abnormalities of Er-DTR at one or more sites. Er-DTR latencies in patients with polyneuropathies were prolonged at all sites compared with controls (P < 0.01). Among patients with various types of polyneuropathies the Er-DTR, mean latencies at all the sites and latency indicative of demyelination (>150% of the normal mean) were higher in patients with DPN than that of CAN or SFN (P < 0.01). Conclusions,,, We conclude that C-DTR are preserved in 35.1% of the patients with polyneuropathies and Er-DTR should be performed in such patients in order to provide electrophysiological evidence of a polyneuropathy. Er-DTR are useful in distinguishing axonal from demyelinating disorders of peripheral nerve, and detection of subclinical involvement of large fibers in SFN. [source] Motor nervous system impairment persists in long-term survivors of childhood acute lymphoblastic leukemiaCANCER, Issue 9 2002Satu S. Lehtinen M.D. Abstract BACKGROUND The objective of the current study was to determine whether therapy for childhood acute lymphoblastic leukemia (ALL) results in long-lasting neurologic signs or electrophysiologic injuries within the motor tracts. METHODS Twenty-seven children who were treated for ALL were studied clinically 5 years after the cessation of therapy by means of motor-evoked potentials (MEPs) elicited by magnetic stimulation transcranially and peripherally. An equal number of healthy children matched with regard to age, gender, and height served as the control group. RESULTS The MEP latencies to the hands and legs elicited by stimulation at the cortex were prolonged significantly in the children treated for ALL compared with the control group, with the differences being 2.2 milliseconds [ms] (P < 0.001) from the cortex to the thenar on the right side and 2.0 ms (P < 0.001) on the left, and 1.4 ms (P = 0.004) from the cortex to the leg on the right side and 1.3 ms (P = 0.004) on the left. Correspondingly, the MEP latency from the fifth lumbar vertebrae (LV) level to the leg also was prolonged, by 1.0 ms (P = 0.005) on the right side and 0.8 ms (P = 0.005) on the left side. The calculated latency between the cortex and the LV level was not found to be significantly longer in those patients treated for ALL compared with the healthy controls. Neurologic signs, in the form of depressed deep tendon reflexes, were observed in 8% of the patients, whereas approximately 33% of the patients were found to have fine or gross motor difficulties and dysdiadochokinesia. CONCLUSIONS Neurologic signs still persisted 5 years after therapy for ALL. Approximately 33% of the patients had fine or gross motor difficulties and dysdiadochokinesia, and demyelinative injuries to the peripheral nerve tracts were found proximally but not within the central nervous system. Cancer 2002;94:2466,73. © 2002 American Cancer Society. DOI 10.1002/cncr.10503 [source] | ||||||||||