S. Shoji

Corticosteroid- responsive asymmetric neuropathy with a myelin protein zero gene mutation.

A patient with hereditary neuropathy presented with asymmetric distal weakness. On nerve biopsy, there was demyelination and onion-bulb formation, and molecular analysis revealed that the patient was heterozygous for an MPZ mutation. The patient improved with corticosteroid treatment.

Ataxic Form of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Clinical Features and Pathological Study of the Sural Nerves

We investigated clinical and pathological features of the sural nerves of 5 patients with the ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and compared these features with those of chronic ataxic neuropathies due to other causes. The CIDP patients presented with slowly progressive ataxia with deep sensory impairment. The durations of the symptoms from onset were relatively short in CIDP (4–8 months) and cancer (3 and 10 months), but long in chronic idiopathic ataxic neuropathy (24–260 months). Corticosteroid therapy elicited a good response in all the patients with CIDP, but a poor response in the patients with other ataxic neuropathies. Sural nerve biopsy of CIDP patients showed a slight or moderate loss of myelinated fiber. This report suggests that ataxic form of CIDP is a steroid-responsive ataxic neuropathy, and large myelinated fibers of the sural nerves in ataxic form of CIDP were better preserved than those in nerves with other chronic ataxic neuropathies.

A Case of Cortical Tremor as a Variant of Cortical Reflex Myoclonus

Myoclonus sometimes represents rhythmical movement and cortical tremor has been defined as a rhythmical movement accompanied by cortical reflex myoclonus [1]. We examined a patient with cortical tremor who demonstrated rhythmical muscle discharges evoked by a peripheral nerve stimulation. A 64-year-old man had a 9-year history of postural tremor involving the hands and fingers. The patient also had generalized seizure, but there were no other neurological findings including dementia. Oral administration of valproate only had the effect of controlling his epileptic attack but had no effect on his tremor; neither had propranolol. Family history involved tremor of the hands, generalized epilepsy or both. Laboratory tests including urinalysis, blood cell count, and serum electrolytes were normal. Thyroid function, lactate and pyruvate levels in serum as well as CSF were all normal. Cranial MRI was normal. Surface EMG of the postural tremor recorded on his forearm revealed rhythmical discharges, which had a reciprocity and in part a co-contraction between antagonists (fig. la). The frequency of mechanical movement, monitored with an accelerometer attached to the hand and estimated through a fast Fourier transformation, was around 7.4 Hz and ranged from 6.3 to 9.4 Hz (fig. lb). EEG showed

Progressive encephalomyelitis with rigidity associated with anti-amphiphysin antibodies

Progressive encephalomyelitis with rigidity (PER) is a rare disorder of unknown aetiology, characterised by muscular rigidity, abnormal postures, painful muscle spasms, and myoclonus, and is caused by inflammation in the brainstem or spinal cord.1,2 We report a case of PER with positive anti-amphiphysin antibodies in the serum and CSF.3 This association has not been previously reported and raises the possibility that PER may have an autoimmune pathogenesis similar to that of stiff person syndrome (SPS).4 ### Clinical features A 37 year old female presented having had symptoms of PER for about three months. Spasms began with several minutes of paroxysmal painful muscle stiffness in the left upper limb, followed by pain and muscle spasms in the upper limbs, shoulders, neck, and back. These spasms were easily evoked by light touches, conversations, and by being startled. The patient remained bedridden and showed left dominant weakness of the limbs, with contractures in the upper limbs and difficulty in relaxing the muscles. She also developed abducent nerve palsy. Her deep tendon reflexes were absent and her plantar responses were both flexor. The serum antinuclear antibody was positive (1:160); …

Acute vocal cord paralysis in hereditary neuropathy with liability to pressure palsies

Hereditary neuropathy with liability to pressure palsies (HNPP) presents with recurrent episodes of peripheral nerve palsies, usually after a minor trauma at the common site of compression or entrapment of the upper and lower extremities.1 Involvement of the cranial nerves has been rare in patients with HNPP, and vocal cord paralysis has not been reported. We describe a patient with HNPP who presented an acute onset of aphonia due to recurrent laryngeal nerve palsy triggered by sleeping in the prone position. A 19-year-old woman presented with two episodes of hand drop and a subsequent episode of aphonia and hoarseness. At age 18 years, she found herself unable to raise her right wrist and extend the fingers of her right hand after waking up from a nap on a train seat, leaning her elbow on the armrest. Although movement of her wrist and fingers recovered somewhat, her paralysis did not recover completely. Six months later, she …

Ataxic form of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)

We reported a 64‐year‐old male with an eight‐month history of gait disturbance and sensory impairment. The patient initially noticed unsteadiness of gait and numbness in his feet, and these symptoms progressed until he was unable to walk without assistance five months later. Vibratory sensation and position sense were markedly diminished, and deep tendon reflexes were absent in all extremities. Motor conduction velocities were slow with prolonged distal latencies, and sensory nerve action potentials (SNAP) were not elicited. Sural nerve biopsy revealed a mild loss of myelinated fibres and segmental demyelination. Cerebrospinal fluid showed normal cell count with protein 526 mg/dL. Anti‐GM1, anti‐GM2 and anti‐GA1 antibodies in serum were positive. We diagnosed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) presenting ataxia. Steroid therapy provided immediate improvement of symptoms and signs. This case suggests that CIDP should be considered as one of the potential causes of ataxic neuropathy.

Reversible ageusia induced by losartan: a case report

Dear Sir, Losartan, a selective antagonist of the angiotensin receptor subtype 1 (AT1), inhibits angiotensin-II-mediated effects and induces hemodynamic changes similar to angiotensin-converting enzyme (ACE) inhibitors (Gradman et al., 1995). Losartan reportedly has fewer adverse effects because the antagonism of angiotensin I receptors is selective. However, Schlienger et al. (1996) described reversible ageusia associated with losartan, and Heeringa and van Puijenbroek (1998) later presented two similar cases. Here, we present the third case report of reversible losartaninduced ageusia. A 69-year-old woman had an 11-year history of spinocerebellar degeneration and a 15-year history of hypertension. She previously had been using amlodipine besilate (5 mg/day) for her hypertension, but the therapy was switched to losartan (50 mg/day) when the amlopidine was found to be causing tachycardia. Two weeks after the initiation of losartan, the patient progressively began to lose taste discrimination. One week later she had complete ageusia, affecting all four primary sensations of taste: sweet, sour, salty and bitter. An examination 1 week later showed no abnormality on the surface of the tongue. The losartan was stopped and replaced by doxazosin mesilate (2 mg/ day), and several days later her sense of taste returned to normal. Drug-induced ageusia is a rare adverse drug reaction most commonly associated with ACE inhibitors, especially captopril and enalapril. McNeil et al. (1979) reported that three of 16 patients treated with the ACE inhibitor captopril developed a loss of taste that persisted until the drug was withdrawn. A variety of other drugs are also known to disturb the sense of taste, including griseofluvin, lincomycin, carbamazepine, transdermal nitroglycerin, D-penicillamine, cefacetril, oxyfedrine and thiamazole (Schiffman, 1983). In cases of taste disturbance caused by thiamazol and D-penicillamine, the problem has been ascribed to the complexing of zinc by these drugs (Erikssen et al., 1975), whilst taste disturbances induced by ACE inhibitors have been tentatively ascribed to the chelation of metal ions, such as zinc (Henkin, 1994). However, the explanation for the development of losartan-induced taste disturbance is not clear. It is important that doctors are aware of the possibility of rare and reversible taste disturbances during the use of losartan.

Horner's syndrome associated with mononeuritis multiplex due to cytomegalovirus as the initial manifestation in a patient with AIDS

We report on a 60-year-old male with AIDS who presented Horners syndrome that was associated with mononeuritis multiplex due to cytomegalovirus (CMV) infection. This is the first case who presented Homers syndrome in the course of AIDS. Horners syndrome associated with mononeuritis multiplex in this patient was the initial manifestation without any opportunistic infections. Since Horners syndrome and mononeuritis multiplex in the present case were both improved by ganciclovir, it is important to consider CMV infection when Horners syndrome or mononeuritis multiplex is observed in immunocompromised patients, such as those with HIV-1 infection or AIDS, even if they do not show any other opportunistic infections.

Acute flank pain, an unusual first symptom of a spinal arteriovenous malformation

This case report describes a 46-year-old man whose first symptom was an attack of acute flank pain, followed by the gradual onset neurological symptoms. We demonstrated a small nidus and serpentine signal-void area in the spinal cord by MRI and diagnosed a glomus type of spinal arteriovenous malformation (AVM). Flank pain is a rare initial finding in an adult with spinal AVM. In cases with acute flank pain, neurologists should consider spinal AVM as a differential diagnosis.