Cervical spondylotic amyotrophy presenting as dropped head syndrome

We report a case of acute-onset dropped head syndrome in a 65-year-old patient in whom the diagnosis of amyotrophic lateral sclerosis (ALS) was initially proposed based on electromyographic signs of neck and shoulder muscle denervation. There were no signs of pyramidal involvement and the clinical and electromyographic signs of motor denervation never evolved beyond the neck and shoulder girdle muscles after a 6-year follow-up period, which argued against ALS. Other causes of dropped head syndrome were carefully ruled out based on clinical findings, electrodiagnostic studies and blood investigations. The restriction of muscle denervation to a few cervical myotomes, the presence of C3–C4 spondylotic changes without associated root or spinal cord compression, and the absence of an alternative explanation to the patient's symptoms strongly supported the diagnosis of cervical spondylotic amyotrophy (CSA). CSA is thought to result from spinal cord microcirculatory disturbances and secondary anterior horn cell degeneration due to ischemia. Our case enlarges the clinical spectrum of focal cervical anterior horn disease, which classically results in more distal monomelic atrophy affecting one or both upper limbs.     

Altered motor axonal excitability in patients with cervical spondylotic amyotrophy

Objective

To investigate the changes in motor axonal excitability properties in cervical spondylotic amyotrophy (CSA).

A case of neuralgic amyotrophy manifesting bilateral anterior interosseous nerve syndrome]

A 49-year-old woman acutely developed severe bilateral shoulder pain followed by weakness of the right shoulder girdle muscles. Within a few days, an inability to flex the terminal phalanges of the bilateral thumbs and index fingers emerged. Neurologic examination 1 month after the onset of symptoms showed atrophy of the right shoulder girdle muscles and mild decreased cutaneous sensation in the distribution of the right axillary nerve. Needle electromyography examination at this time showed fibrillation potentials in the right deltoid and bilateral flexor pollicus longus muscles. Recruitment of the right deltoid, supra- and infraspinatus muscles was reduced. Motor unit potentials in these muscles were of normal configuration. Nerve conduction studies in the upper limb were normal. She was diagnosed as neuralgic amyotrophy with bilateral anterior interosseous nerve syndrome. 4 months later, the muscles innervated by the bilateral anterior interosseous nerve improved in the muscle strength. Clinical features of this case were compatible with a mononeuropathy multiplex form of neuralgic amyotrophy associated with an autoimmune etiology. We think this case is important for speculating the pathogenesis of neuralgic amyotrophy. This case reminds us that patients with neuralgic amyotrophy sometimes demonstrate anterior interosseous nerve syndrome and most patients manifesting anterior interosseous nerve syndrome are patients with neuralgic amyotrophy.     

A case of hypersomnia resembling Kleine-Levin syndrome

A case report is presented of episodic hypersomnia with bouts of several days duration. The authors discuss various clinical syndromes with sleep disorders with particular consideration of the Kleine-Levin syndrome.     

A case of neuralgic amyotrophy with antiganglioside antibody]

A 54-year-old-man experienced pain from his left shoulder to his left arm and had difficulty in lifting his arm after a febrile episode. Three weeks after the onset, he was admitted to our hospital. Neurological examination demonstrated weakness and atrophy of the left deltoid muscle. Deep tendon reflexes were normal and no pathological reflexes were elicited. CSF total protein was slightly increased. The occurrence rate of F-waves was decreased in the left upper limb. Magnetic resonance imaging (MRI) study of the cervical cord and brachial plexus with and without Gadolinium infusion showed no abnormalities. Serological study showed that IgM anticytomegalovirus antibody was positive, and that serum IgM anti-GalNAc-GD1a antibody and IgM anti-GM2 antibody were positive. Symptoms were improved after treatment with mecobalamin, 1.5mg/day. This case was considered neuralgic amyotrophy after cytomegalovirus infection. The antiganglioside antibodies may play some role in its pathogenesis.     

Tract pain because of cervical spondylotic myelopathy: A case series

Spinal cord compression may lead to pain that is sometimes directed to areas far below the compression level. In certain cases, it may present as sciatica pain, knee pain or low back pain (LBP). These types of pain are called tract pain or funicular pain. Tract pain because of cervical spondylotic myelopathy (CSM) may lead to delays in the diagnosis and treatment of CSM in some cases, and sometimes unnecessary medical and surgical treatments. This study evaluated the results of four patients who presented to the outpatient clinic with complaints of LBP accompanying CSM findings. This study aims to present the improvement in low back pain as a result of anterior cervical microdiscectomy and cage procedure in four patients who presented with tract pain because of CSM, which is a rare condition.     

A case report of an intramedullary cervical teratoma in an adult patient with cervical spondylotic radiculopathy

Spinal cord teratomas are rare. There are few reports of teratomas affecting the cervical spine and their association with spondylotic radiculopathy has not been described. A 59-year-old woman with history of fecal incontinence attended with cervical radicular pain radiating to upper limbs. Physical examination showed distal muscle hypotrophy and abolishment of bicipital, tricipital, and brachioradialis reflexes of the right arm, preserving proximal strength. Also, hiporreflexia and loss of proprioception in the right lower limb was observed. Magnetic resonance imaging showed an intramedullary mass at C7-T1, accompanied by intervertebral disk protrusions and dural sac compressions at the same level. One-stage posterior–anterior operative approach for tumor resection, decompression of the radiculopathy and replacement of intervertebral discs was performed. The histopathological diagnosis was for a mature teratoma. We described the first case of an intramedullary cervical teratoma associated with radiculopathy in an adult, providing evidence of rare long-lasting teratomas affecting the cervical spine.     

A case of chronic demyelinating polyneuropathy resembling the Guillain-Barré syndrome

A case of demyelinating polyneuropathy is reported which was clinically and pathologically indistinguishable from the Guillain-Barré syndrome, with the exception that the symptoms progressed steadily for 7 months. Pathologically, the peripheral nerves and intraspinal roots showed selective demyelination with axonal sparing and patchy mononuclear inflammation.The posterior columns of the spinal cord also showed selective demyelination with axonal sparing. The simultaneous destruction of central and peripheral myelin is discussed in relation to the immune theory for the Guillain-Barré syndrome. Numerous anterior horn cells showed severe chromatolysis although there was little axon loss. It is suggested that chromatolysis may occur after severe and longstanding demyelination.     

Motor unit number index (MUNIX) in the quantitative assessment of severity and surgical outcome in cervical spondylotic amyotrophy

ObjectiveTo assess the feasibility of motor unit number index (MUNIX) in the quantitative assessment of the cervical spondylotic amyotrophy (CSA).MethodsMUNIX was recorded bilaterally on the abductor pollicis brevis, abductor digiti minimi, biceps brachii and middle deltoid in 41 normal controls and 47 patients with CSA (distal-type to proximal-type ratio: 25 to 22). Additionally, patients were assessed on handgrip strength (HGS), the disabilities of arm, shoulder and hand (DASH) and Medical Research Council (MRC) scales. These examinations were re-evaluated approximately 18 months after surgery in 37 of these CSA patients.ResultsMUNIX values were noticeably lower in the mainly affected muscles of CSA patients than those in controls (P < 0.05), and 49.0% (51/104) of the tested muscles with abnormal MUNIX measurements showed normal muscle strength. Significant correlations between MUNIX measurements and both DASH and MRC scores were observed in both CSA patient groups (P < 0.05). Postoperative longitudinal follow-up analysis identified significant increase in motor unit number in both CSA patient groups within approximately 18 months (P < 0.05), with or without improved measures of motor function.ConclusionsA significant reduction in MUNIX values related to motor impairment was observed in CSA patients, even in the subclinical stage. Compared to measures of motor function, the MUNIX measurements in the patients with CSA improved more noticeably after surgical intervention.SignificanceMUNIX may serve as an available supplementary test to quantitatively evaluate the motor dysfunction in CSA and to track its progression, that is complementary to conventional electromyography.     

A case of myasthenia gravis with neurogenic amyotrophy

Summary A 60-year-old woman affected by myasthenia gravis with onset at 27 years is described. At the age of 54 years the patient showed amyotrophy of the shoulders and of the legs with bilateral steppage. There was a gradual worsening and progression of the atrophy to other muscles. The nature of the disease is discussed following EMG and histologic data.

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Zusammenfassung Es wird eine 60jährige Frau, die an Myasthenia gravis leidet, beschrieben. Die Krankheit begann, als sie 27 Jahre alt war. Mit 54 Jahren zeigte die Patientin eine Myatrophie der Schulter und der Beine mit bilateralem Steppengang. Zunehmende Verschlechterung und Übergreifen der Atrophie auf andere Muskelgebiete. Die Natur der Krankheit wird auf der Basis der EMG und der histologischen Befunde besprochen.

     

A case of McLeod syndrome]

A 52-year-old man was admitted to our hospital in 1997 because of high serum creatine kinase (CK) level. Neurologic findings revealed chorea on the trunk and extremities, mild weakness of proximal muscles, and absence of deep tendon reflexes in four extremities. Serum CK was elevated to 3,494 U/l (normal, below 235). A peripheral blood smear showed acanthocytes in approximately 1% of the red blood cells (RBCs). Very weak expression of the Kell antigens (K2, K4, K5, and K7) on his RBCs led us to make a final diagnosis of McLeod syndrome. Muscle biopsy from the left biceps showed increased variability in fiber diameter, a few regenerating fibers, scattered fibers with internal nuclei, and mild fiber type grouping. Immunohistochemical analyses of dystrophin. merosin, and adhalin were normal. Although McLeod syndrome is a rare X-linked recessive disorder, it is clinically important for differential diagnosis of chorea acanthocytosis and hyperCKemia.     

A case of Steel-Richardson-Olszewski syndrome]

A case of Steele-Richardson-Olszewski disease is reported calling attention to diagnostic difficulties in cases of chronic dementia with neurological signs. The authors propose including therapy with antiviral and antiparkinsonian drugs in S-R-O disease. The presented case confirms the effectiveness of this treatment.     

A case of acute cervical epidural abscess]

Spinal epidural abscess (SEA) is a rare condition with potentially devastating consequences and more aggressive diseases than previously recognized. We report a case with cervical epidural abscess treated successfully by emergent neural decompression. A 49-year-old man presented with fever and cervical pain. An magnetic resonance imaging (MRI) showed an epidural abscess at C5-6. His condition deteriorated with the development of complete sensory loss and tetraparesis. Emergent anterior neural decompression was performed, and pus was aspirated which grew Staphylococcus aureus. Adequate antibiotics were administered, and the patient showed remarkable improvement of neurological signs and symptoms. He underwent reconstruction surgery 3 weeks after the first operation, and returned to his original job 3 months after the operation. Prompt diagnosis and satisfactory surgical decompression are essential to avoid irreversible neurological sequelae in epidural abscess of the cervical spine.