Neck rigidity as a physical manifestation of higher brain hypo-function/dysfunction.

A total of 308 patients, who received inpatient rehabilitation in department of Neurology in Sapporo City General Hospital, were studied. The patients were divided into four subgroups according to the presence or absence of Parkinsonism and the location of organic neurological lesions: group A consisted of 47 patients with brain lesions who presented with Parkinsonism; group B consisted of 135 patients with manifestations deriving from brain disorders other than Parkinsonism; group C consisted of 68 patients with manifestations deriving from spinal cord, peripheral nerve, and muscle disorders; and group D consisted of 58 patients with non-organic functional disorders and patients with organic lesions not responsible for clinical manifestations. The patients were administered a battery of 5 tests for evaluating frontal lobe and/or higher brain function. In result, our study confirmed higher brain/frontal lobe dysfunction to affect the grade of neck rigidity unless there is an interruption, in the control of peripheral organs, by the brain. Consequently the association between higher brain/frontal lobe dysfunction and the grade of neck rigidity were confirmed. Although neck rigidity may be a minor clinical manifestation, it can provide important clues to brain function.

Predominant sensory ataxic neuronopathy showing marked improvement after resection of a thymoma followed by intravenous immunoglobulin therapy.

A 64-year-old man was admitted to our hospital because of difficulty in walking and numbness in his lower extremities. Upon investigation, the patient was diagnosed as having predominant sensory ataxic neuronopathy associated with thymoma. Surgical resection of the thymoma followed by intravenous immunoglobulin therapy resulted in marked improvement of the patient's clinical symptoms; therefore immunological mechanisms related to the presence of the thymoma were suspected to underlie the neuropathy in this patient. We did not find any previous reports of an association of sensory ataxic neuronopathy with thymoma, even after a thorough search of the literature.

Smooth-pursuit eye movement--a convenient bedside indicator for evaluating frontal lobe and intellectual function.

We hypothesized that smooth-pursuit eye movement is related to higher brain functions and that its impairment (iSPEM) could be useful in diagnosing neurological dysfunctions. We prospectively examined 305 patients and studied the relations between iSPEM and five items that reflect intellectual and/or frontal lobe function (dementia, sucking, snouting, hand grasping, elbow flexion response). We divided these patients into four subgroups: group A, patients with cerebellar ataxia as the presenting manifestation and with main lesions in the cerebellum; group B, patients with main lesions in brain regions other than the cerebellum; group C, patients with main lesions in the spinal cord, peripheral nerves, and muscles; and group D, patients with non-organic functional disorders, such as paroxysmal attacks and physical pain. Consequently, iSPEM was significantly (p<0.01) related to impairment in intellectual and frontal lobe functions in patients with cerebral lesions, and it also can be regarded as being equivalent to primitive reflexes.

Absence seizures with myoclonic seizures as an early manifestation of dentato-rubro-pallido-luysian atrophy (DRPLA): a follow-up clinical course of twelve years.

Typical absence seizures and isolated myoclonic seizures are both classified as age-related generalized seizures and are considered to be benign neurological manifestations. Concomitance of the two types of seizure is considered benign if it does not accompany other types of seizures or other neurological problems. We followed up a ten-year-old girl with isolated absence and myoclonic seizures whose family history of mental and neurological signs was initially not disclosed. After several years, the family history of neurological and mental problems was finally disclosed, and the diagnosis of dentato-rubro-pallido-luysian atrophy (DRPLA) was confirmed. The patient's clinical course was slowly progressive, and by age 21 she was in a nearly vegetative state. We would like to alert clinicians to consider DRPLA when diagnosing patients with absence and/or myoclonic seizures, even when they present the clinical features of benign epilepsies in the early stage.

Malignant lymphoma originating in the cauda equina mimicking the inflammatory polyradiculoneuropathy.

A 67-year-old woman was diagnosed with inflammatory polyradiculoneuropathy. The intravenously administered immunoglobulin (IVIG) treatment that she received several times over a 3-year period relieved her clinical symptoms of muscle weakness and sensory disturbances, but these symptoms had worsened thereafter despite further IVIG treatment. MRI detected a solid tumor involving the cauda equina and pathological examinations confirmed this to be malignant lymphoma. The clinical and radiological findings for the malignant lymphoma of the cauda equina in this patient were quite similar to those for the inflammatory polyradiculoneuropathy.

Hodgkin's disease-related central nervous system angiopathy presenting as reversible posterior leukoencephalopathy.

Reversible posterior leukoencephalopathy syndrome (RPLS) is a clinical syndrome characterized by headache, conscious disturbance, seizure, and cortical visual loss with neuroimaging finding of edema in the posterior regions of the brain, with a reversible course (1). The precise pathomechanism of RPLS is not understood. However, association with uncontrolled hypertension, renal failure, eclampsia, or immunosuppressive agents has been implicated (1). We describe herein a case of Hodgkin's disease (HD)-related central nervous system (CNS) angiitis with neuroimaging finding suggestive of RPLS. The pathophysiology of RPLS in cases with CNS angiitis is discussed.

Elbow flexion response as another primitive reflex.

In daily clinical practice we noticed that patients with intellectual impairment spontaneously flex the elbow within a few seconds of the forearm being manipulated during routine examination of spasticity of the muscles in the upper extremities. We termed this phenomenon elbow flexion response (EFR), and prospectively studied it in 229 patients who underwent in-hospital rehabilitation following brain damage. Evaluation of each patient included EFR, patient profile, ability to communicate, scores on three parameters from various intelligence tests, scores on seven parameters testing primitive reflexes, and scores on three parameters describing personality. We investigated for relationships among these parameters. Consequently, although EFR rarely have a statistical association with the varied profiles of patients, patients with bilateral lesion or bilateral paresis demonstrated significantly more marked EFR than those with unilateral lesion or unilateral paresis. Patients with involvement of the frontal lobe showed significantly more marked EFR than those without damage in this area. Elbow flexion responses occurred significantly more frequently in relation with lower scores on intelligence and occurred with significantly higher frequency in conjunction with the more marked appearance of conventional primitive reflexes. Therefore, we conclude that EFR have a strong association with intelligence and with the existence of frontal lobe lesion, and their mode of clinical presentation parallels that of primitive reflexes particularly that of the grasp reflex. We propose that EFR could be referred as a variation of the grasp reflex occurring in the more proximal or axial part of the body.

[A case of Ramsay Hunt syndrome initiated with hoarseness and dysphagia: consideration on spreading mechanisms of cranial neuropathy].

A 85-year-old woman was admitted to our hospital because of progressive hoarseness and dysphagia of two days' duration. Neurological examination on admission revealed right pharyngeal and vocal cord palsies. After admission, gradual swelling of her right ear was noted, and on day 6, vesicular eruptions in her right geniculate zone, the right VII and the VIIIth cranial nerve palsies were added. On the basis of these findings, she was diagnosed as Ramsay Hunt syndrome. Varicella zoster virus (VZV) infection was confirmed by the elevation of serum anti-VZV-antibody titer, and detection of VZV DNA from cerebrospinal fluid. Ramsay Hunt syndrome associated with multiple cranial neuropathy is not frequently reported. Reviewing Japanese literatures, we found that the IX and the Xth cranial nerves were most frequently affected, and the half of these cases were initiated with cranial neuropathy other than the VIIth. Additionally, spreading mechanisms of cranial neuropathy, and the early diagnostic problems of these conditions were discussed.