دکتر بهرام حقی آشتیانی

Amyotrophic lateral sclerosis (ALS) is an adult-onset motor neuron disease with a poor prognosis that leads to limb and or bulbar muscle degeneration. Several demographic and biological factors have prognostic importance, but little data exist on the relationship between clinical, electrodiagnostic, and laboratory markers as predictors of disease progression. We aimed to assess the relationships between different aspects of ALS patients’ clinical, electrodiagnostic, and laboratory features and their level of functioning. 

We included 27 ALS patients diagnosed within the last two years. A neurology resident conducted clinical assessment and electrodiagnostic studies. The motor unit number index (MUNIX) and compound motor action potential (CMAP) were used to measure motor unit loss. Serum creatinine, urea, albumin, and creatine kinase were measured as laboratory markers. We used the Persian version of the ALS functional rating scale (ALS-FRS) as the main outcome measure. The Pearson correlation coefficient was calculated to assess the correlations using the SPSS software, version 16.

None of the demographic or laboratory parameters were correlated with ALS-FRS. Patients with the onset of disease in the limbs had a higher MUNIX score than those with bulbar onset. Also, increased body mass index (BMI) was associated with lower CMAP and MUNIX scores (P=0.02). Higher serum creatinine levels were significantly associated with higher lower limb MUNIX (P=0.04). Higher lower limb MUNIX was associated with a higher lower limb functional score. 

Decreased serum creatinine may indicate lower limb motor unit loss in patients with ALS. Also, MUNIX scores may be used as substitutes for ALS-FRS in ALS trials. Further research is needed to elucidate the clinical application of these findings.

Carpal tunnel syndrome (CTS) is the most prevalent entrapment syndrome in the upper limbs, for which pregnancy is a known risk factor. CTS diagnosis is confirmed via nerve conduction studies (NCSs), which sometimes is expensive, and the electrical stimulation makes it an unpleasant diagnostic modality, especially for pregnant subjects. Recently, high-frequency ultrasonography (HF-USG) is known as a diagnostic method. This study is concerned with determining the diagnostic value of this modality for CTS among pregnant women.

This cross-sectional case-control study was conducted with 40 CTS cases and 40 matched controls. The HF-USG of wrists was performed bilaterally on all participants with a focus on the median nerve cross-sectional area (MNCSA) at the carpal tunnel (CT) inlet.

Mean MNCSA was statistically different between the CTS group (11.71 ± 1.86 mm2, range: 8 to 18 mm2) and the control group (6.75 ± 1.38 mm2, range: 4 to 11 mm2) (P < 0.001). The receiver operating characteristic (ROC) curve was drawn, and the cross-sectional area (CSA) cut-off point of 8.5 mm2 showed sensitivity and specificity of 98% and 93%, respectively. The positive predictive value (PPV) and the negative predictive value (NPV) were 95% and 98%, respectively, with the mentioned point as the diagnostic threshold.

HF-USG of the median nerve can be utilized as a preferable alternative to NCS (the current gold standard diagnostic method) in pregnant women, due to its convenience and lower cost, or at least, it can be used as a screening tool among pregnant women with suspicious symptoms.

Amyotrophic Lateral Sclerosis-Specific Quality of Life-Revised (ALSSQOL-R) encompasses 50 items which assess quality of life (QOL) in patients with amyotrophic lateral sclerosis (ALS) in six major domains. This study aims to translate the ALSSQOL-R into Persian and evaluate its reliability and validity among Iranian patients.

ALSSQOL-R was translated by the standard multi-step forward-backward method. Content validity was calculated using item content validity index (I-CVI). Three items in the “intimacy” domain were deleted considering Iranian culture. Cronbach’s alpha was used for all 6 dimensions to calculate the internal consistency reliability. Test-retest reliability was evaluated using intraclass correlation coefficient (ICC) with one-month interval. Concurrent validity was measured by the validated version of 36-Item Short Form Health Survey (SF-36) questionnaire.

Sixty-three patients with ALS were enrolled in the study. I-CVI was 70%, promoted to 85% after modifications (acceptable). Regarding internal consistency reliability, Cronbach’s alpha in all six domains was ³ 0.70 and total Cronbach’s alpha was 0.89 which is assumed as good. In terms of test-retest reliability, ICC [95% confidence interval (CI)] was 0.91 (91%) and Pearson correlation coefficient (r) was 0.90 (P < 0.001), all indicating an excellent reliability. The concurrent validity was established based on a strong correlation with SF-36 (r = 0.744, P < 0.001).

The findings show that the modified Persian version of ALSSQOL-R is a valid and reliable QOL questionnaire which can be used for Iranian patients with ALS in both clinical and research settings.

Few studies have reported the association of Guillain-Barre syndrome (GBS) and coronavirus disease-2019 (COVID-19) infection. In this study, we reported GBS in six patients infected with COVID-19 and reviewed all existing literature about GBS in association with COVID-19.

This study was performed in three referral centers of COVID-19 in Iran, and six patients with the diagnosis of GBS were enrolled. Patients enrolled in the study with acute progressive weakness according to the demyelinating or axonal variant of GBS, according to Uncini's criteria.

Four of our patients had axonal polyneuropathy, two patients had demyelinating polyneuropathy, and one patient required mechanical ventilation. All our patients had a favorable response to treatment. In one patient, the GBS symptoms recurred four months after the first episode.

Limited case reports suggest a possible association between GBS and COVID-19. Such associations may be an incidental concurrence or a real cause-and-effect linkage; however, more patients with epidemiological studies are necessary to support a causal relationship.

We aimed to compare the sonographic measurement of median nerve cross-section area (CSA) in patients with Amyotrophic Lateral Sclerosis (ALS) and healthy individuals. The effect of duration of the disease on correlations between paraclinical findings and ALS functional rating scale (ALSFRS) were secondarily aimed to be evaluated. The cross-sectional study was approved by the Ethical Committee of Iran University of Medical Sciences and conducted between January 2017 and December 2018. We evaluated the median nerve surface area by means of sonography in 35 ALS patients and 35 healthy controls. Compound muscle action potential (CMAP) amplitudes during nerve conduction study and ALSFRS were recorded by the same trained specialist. Data were analyzed using SPSS software version 18. We did not find a significant difference between CSA in ALS patients and the normal population (P>0.05). Comparing to normal individuals, the mean CMAP decreased significantly in ALS patients (6.6±3.07 mV versus 10.25±2.2 mV, P<0.001). ALSFRS correlated with both CSA of the median nerve at the wrist (P:<0.001, r:0.78) and the CMAP (P:<0.001, r:0.74) that were confirmed by regression models designed to consider the effect of disease duration on these correlations. CSA was not different between ALS patients and the normal population, but CMAP decreased in ALS patients. ALSFRS correlated with both CSA and CMAP of the median nerve.

Meningeal melanocytoma is a rare tumor of nervous system, which originates from leptomeningeal melanocytes. The locations of melanocytoma in the nervous system are most frequently in the posterior fossa or along the spinal cord, and usually appear as an extra-axial mass. The manifestations of tumor are most often due to its compressing effect on adjacent nervous structures that causes various neurological signs and symptoms depending on its locations. It may also cause superficial siderosis of the central nervous system [1]. In this case we describe another manifestation of this tumor which raised intracranial pressure and developed its neurological signs and symptoms. The patient was a 33-year old man with a two-year history of headache and tinnitus, transient diplopia, and had also a three- month history of progressive bilateral visual and hearing loss. The medical investigations of the patient reveal raised intracranial pressure (RICP) with a high concentration of protein in the cerebrospinal fluid, and an extra-axial mass at the T11-12 level in magnetic resonance imaging of the spinal cord. The patient underwent surgical removal of the tumor, in which the pathological study characterized the tumor as a meningeal melanocytoma. After surgery the CSF pressure returned to normal state, and its protein level decreased. The patient's hearing loss improved significantly but the visual defect did not change. Base on various causes of the RICP, especially when there is abnormality in CSF protein without any known cause, we must consider melanocytoma as a treatable cause, and thus in such patients, performing spinal cord magnetic resonance imaging (MRI) is a valuble technique for diagnosis as well as investigation.

To improve the debilitating features of Parkinson disease (PD) different medical and surgical approaches are available. Subthalamic nucleus deep brain stimulation (STN-DBS) was appeared to be a promising method during last two decades. This study aimed to evaluate early motor outcomes of this procedure in first trial of Iranian patients.

Thirty-seven consecutive patients with advanced Parkinson disease with poor response to common medical agents underwent bilateral STN-DBS. For assessment of motor function parameters Unified Parkinson Disease Rating scale III (UPDRS III) was used. We compared total scores and subscores in three measurements performed as 1) preoperative off-medication, 2) preoperative on-medication and 3) six months postoperative on stimulation and on medication. Reduction in drug consumption was assessed with regard to administered doses of L-Dopa before and after surgery in stable states.

26 men and 10 women with mean age of 50 years were evaluated (one person expired before 6-month follow-up). Mean total scores of UPDRSIII were calculated as 5.2±54.52, 2.88±18.22 and 3±12.8 in three measurements, respectively (p=0.003). PostHoc analyses showed significant improvement among all measurements. Analysis of subscores also revealed significant amelioration in rigidity, resting tremor, hand movement, leg agility, finger tap and rapid alternating movement in on-medication phases of pre- and post-operation (all with p<0.01). The mean administered L-Dopa were224±1296 mg/d and 174±782 mg/d before and after surgery, with significant decline (p<0.001) in administered L-dopa dose.

The results indicate that bilateral STN-DBS can lead to significant short-term improvement of the motor symptoms especially in some debilitating symptoms such as rigidity and tremor in advanced PD. It also accompanies with remarkable reduction in needed doses of drugs. The findings support other studies with similar follow - up interval; however, continuous evaluations are needed for long-lasting effects.