Minimal Muscle Weakness Research Articles

A Case Report of Sensory Guillain-Barré Syndrome with Prominent Autonomic Dysfunction in a Systemic Lupus Erythematosus Patient

Sensory Guillain-Barré syndrome (GBS) is a rare heterogeneous subgroup of GBS characterized by the primary involvement of sensory neurons resulting in a distinctive clinical presentation. Sensory GBS usually occurs with acute and monophasic sensory symptoms, and no or minimal muscle weakness. Sensory GBS patient show hypo- or areflexia, distal paresthesia, and normal cerebrospinal fluid finding or albumino-cytologic dissociation which are suggesting that these conditions are a GBS variant. Autonomic dysfunctions have rarely been reported in sensory GBS patient presenting with postural hypotension, abnormal heart rate response to respiration. In this case, we demonstrate a patient with autonomic symptoms dominant sensory GBS in systemic lupus erythematosus.

Comparison of Genicular Nerve Block in Combination With Adductor Canal Block in Both Primary and Revision Total Knee Arthroplasty: A Retrospective Case Series.

The management of pain in patients undergoing total knee arthroplasty (TKA) for chronic knee osteoarthritis (OA) has remained a challenge for the anesthesiologist regarding regional anesthesia as no single regional technique is adequate with regard to balancing effective analgesia with minimal muscle weakness. Severe postoperative pain following TKA has been shown to negatively impact patient outcomes and mortality. The genicular nerve block has recently been demonstrated to provide effective analgesia to the anterior and posterior knee capsule in recent studies. In this retrospective case series, we compare the efficacy of combined genicular nerve block (GNB) and adductor canal block (ACB) to only ACB in both primary and revision TKA patients. This combined novel approach for TKA patients can be utilized to improve patient pain scores and early ambulation, limiting the use of opioids and early discharge.

Clinical and serological features of acute sensory ataxic neuropathy with antiganglioside antibodies

There is as yet no consensus for considering pure acute sensory ataxic neuropathy (ASAN) as a variant of Guillain-Barré syndrome (GBS). Reactivity against gangliosides sharing disialosyl epitopes has been reported in these patients. The aim of this study was to determine the spectrum of reactivity against gangliosides in ASAN and to define the clinical pattern. From our database we identified patients with suspicion of ASAN. We defined ASAN as the presence of ataxia of peripheral origin with loss of proprioception, and areflexia, absence of ophthalmoplegia and no or minimal muscle weakness. Patients who met these criteria were retrospectively reviewed for their spectrum of reactivity against gangliosides and clinical features. We identified 12 patients fulfilling pre-defined criteria for ASAN. Reactivity against gangliosides containing disialosyl epitopes was present in seven patients. Concomitant reactivity against other gangliosides was present in 6/7 patients. All patients presented good prognosis and an antecedent illness was present in nine. Our results support the previously described clinico-immunological association between ASAN and disialosyl specificity, and widen the spectrum of reactivity against gangliosides. The acute presentation with a monophasic course, and good prognosis in all cases, together with transient immunoglobulin G antiganglioside antibodies and infectious antecedent in 7/12 patients support the inclusion of ASAN as a GBS variant.

２０歳代で歩行不能となった肢帯型筋ジストロフィー２Ｍ型の１症例

We report a 29-year-old man with limb-girdle muscular dystrophy type 2M (LGMD2M) caused by a compound heterozygous mutation of 3-kb insertion in the 3'-untranslated region and c.1073A > C (p.Q358P) mutation in exon 9 in FKTN. He had been diagnosed since childhood as having Becker muscular dystrophy based on limb-girdle muscle weakness and calf muscle hypertrophy. Loss of ambulation occurred at age 26 years and cardiomyopathy was noted one year later. Muscle biopsy at age 29 revealed dystrophic changes with loss of immunoreactivity to alpha-dystroglycan (alpha-DG), which prompted us to analyze FKTN and subsequent establishment of the diagnosis of LGMD2M. Brain MRI revealed hypoplasia of the right cerebellar hemisphere and tonsil. Dysplastic part was present in the lower medial part of the hypoplastic hemisphare, which was bordered by a deep cleft. Previously reported LGMD2M patients had mild or minimal muscle weakness in addition to dilated cardiomyopathy. In contrast, our patient had more severe skeletal muscle weakness and loss of ambulation. Treatment with 3-blockers or angiotensin II converting enzyme blockers has been reported to be efficacious for cardiomyopathy in patients with muscular dystrophy. The precise diagnosis should be established early in patients with muscular dystrophy complicated with cardiomyopathy.

EM.P.2.07 Frequency of fukutinopathy in Japanese cardiomyopathy patients with myopathy

Background: FKTN is a responsible gene for Fukuyama-type congenital muscular dystrophy, which is characterized by severe congenital muscular dystrophy with brain anomalies. We previously reported that FKTN mutations also can cause dilated cardiomyopathy. These patients showed normal intelligence, minimal muscle weakness, and high serum CK level. On the other hand, we recently reported low incidence of fukutinopathy among patients with idiopathic cardiomyopathy, raising a possibility that fukutinopathy may be associated with a cardiomyopathy-plus-myopathy phenotype but not with a cardiomyopathy-only phenotype.

Dermatomyositis/polymyositis associated with malignancy: our experience with ten patients and review of relevant literature

AbstractObjective: To analyse the clinical and laboratory data of patients diagnosed with malignancy associated dermatomyositis/polymyositis (DM/PM) seen between January 2002 and February 2007.Methods: The demographic, clinical, treatment and follow‐up data from the case records of all 10 patients diagnosed with malignancy‐associated inflammatory muscle diseases (IMD) namely, DM/PM, were entered in a proforma and analysed.Results: In patient nos. 1–8, malignancy was diagnosed prior to the diagnosis of myositis; in patient no. 9, it was detected during screening and patient no. 10 developed breast cancer 5 years after PM diagnosis. All the patients had typical clinical and laboratory features of IMD, except two patients, who had amyopathic dermatomyositis with predominantly cutaneous features, creatine kinase levels less than two times the upper limit of normal and minimal muscle weakness. Breast cancer (6/10) and ovarian cancer (3/10) were the most commonly associated malignancies. All but one (patient no. 8) responded to treatment. Malignancy relapsed in four patients with concurrent relapse of myositis in three. These three patients died of the malignancy. One patient was lost to follow‐up. The mean duration of follow‐up in others was 21 months (range 12–60 months).Conclusion: DM is more commonly associated with malignancy. Carcinoma of the breast was the most commonly associated malignancy. Myositis responded in most patients. Death was due to the underlying malignancy. In most cases, the two conditions occur within 1 year.

Percutaneous Endoscopic Discectomy for Far Lateral Lumbar Disc Herniations: Prospective Study and Outcome of 66 Patients

Extraforaminal disc herniations represent up to 11% of all lumbar herniated discs. Numerous surgical approaches have been described. Percutaneous endoscopic discectomy (PED) is one of the minimally invasive techniques; after mastering this procedure it is a practical method that is used for treatment of foraminal or extraforaminal disc herniation. The outcome of PED for treatment of foraminal or extraforaminal disc herniation has been studied. A total of 66 patients with foraminal or extraforaminal lumbar disc herniation was treated by applying the PED technique between January 1998 and June 2005. The positions of the herniated disc levels were L2-3 (n=5, 8%), L3-4 (n=19, 28%) and L4-5 (n=42; 64%). The selected patients had no previous surgery, appropriate conservative therapies were done before the operations, and MRI was the main diagnostic method with the clinical findings. Evaluation of the patients with clinical examinations, visual analogue pain scale (VAS) and Oswestry scale was performed preoperatively, on postoperative day 7 and in the postoperative 6-12 months period. In two patients (n=1, L4-5 and n=1, L3-4) disc material could not be removed with PED, so discectomy was performed with microscopic visualization during the same session. Three patients (n=3, L4-5) were reoperated on three to six months after primary surgery due to recurring disc problems with microscope visualization. In two patients (n=2, L4-5) root nerves were partially damaged, and in two patients (n=2, L4-5) root nerves were impinged by the working channel. These 4 patients had dysesthesias from just after surgery to a mean of 45 days after surgery. One of recurrent cases was among these patients. Neurological examinations showed minimal muscle weakness of the quadriceps femoris and diminished sensation of the L4 dermatomal area in patients with partial nerve root damage. This patient improved and the neurologic examination became normal with disappearance of the dysesthesia. There was no sign of reflex sympathetic dystrophy (RDS). With these two patients VAS and Oswestry scales scores decreased significantly early in the postoperative follow-up. The postoperative 6-month average scores are favourable in comparison with the average score at postoperative day 7. The postoperative 12-month scores showed no significant differences to those of postoperative month 1. Percutaneous endoscopic discectomy is a minimally invasive method and offers many benefits to the patient, but extensive surgical practice is needed to become a capable surgeon. Consequently this technique can only be a treatment option on appropriate patients. This study reconfirmed that the removal of fragmented disc material is achieved and offers a pain-free status.

Combined Spinal and Epidural Analgesia in a Parturient With Severe Myasthenia Gravis

Background and ObjectivesA 27-year-old woman with a history of myasthenia gravis presented for vaginal delivery.MethodsA combined spinal epidural was initiated with 7.5 μg intrathecal sufentanil at the time of first request for analgesia followed by 140 mg epidural lidocaine prior to spontaneous vaginal delivery.ResultsThe patient had a pain-free spontaneous vaginal delivery uncomplicated by muscle weakness.ConclusionCombined spinal and epidural anesthesia using intrathecal opioids initially provides labor analgesia with minimal muscle weakness in the parturient with myasthenia gravis.

Scoliosis associated with central core disease

A 12-year-old Japanese girl who had progressive severe scoliosis but with minimal muscle weakness in the extremities was found to have central core disease. In her muscle biopsies obtained from the biceps brachii and paraspinous muscles, there was type 1 fiber atrophy and predominance, as is commonly seen in congenital myopathies, but the core structure was identified only in the former. To determine whether scoliosis is a prominent feature of this disease, we reviewed 10 patients with central core disease in our laboratory and found 6 ambulant patients who had mild-to-moderate scoliosis. Since kyphoscoliosis becomes prominent as muscle weakness progresses to loss of ambulation in most muscle diseases, this disproportionate spinal involvement in central core disease appears to be a striking feature. All patients with ‘idiopathic’ scoliosis deserve a careful neurological evaluation, even if they have minimal muscle symptoms in the extremities.

An etiologic study of clinical paralytic poliomyelitis.

Laboratory evidence of poliovirus and various non-poliovirus infections found in 358 cases clinically diagnosed as paralytic poliomyelitis was examined with respect to the severity of paralysis and to prior immunizations with killed poliovirus vaccine. Poliovirus infection was confirmed in 85% of patients with severe spinal paralytic disease but in only 21% of those with minimal muscle weakness. Non-poliovirus infections were associated with one-third of the cases with minimal weakness, less than 10% of those with moderate paralysis and not at all with cases of severe paralysis. In each category of paralysis poliovirus infection was less often confirmed among patients who had been immunized, whereas non-poliovirus infections were found more often in the vaccinated patients. Altogether a non-poliovirus was implicated, and poliovirus infection excluded, in 41 cases.