Hypokalemic thyrotoxic paralysis: a rare cause of tetraparesis with acute onset in Europeans

We describe a 21-year-old Italian male affected by hypokalemic tetraparesis with acute onset. In the emergency ward, the patient was agitated, with tachycardia (140/min) and systolic hypertension (180/70 mm Hg). He was not able to flex the lower extremities against a light resistence and furthermore, he was hypotonic and without tendon reflexes. One hour later he developed strength deficit of the upper extremities as well. Biochemical analyses revealed severe hypopotassemia (2.1 meg/l). After administration of 140 meq potassium phosphate, the patient began to improve, and 12 h after the onset he was able to walk normally. Successive investigations documented an undiagnosed case of Graves' disease. Thyrotoxic hypokalemic paralysis has been observed almost only in Asians, however, with this case and others reported, we believe that it should be considered as a cause of muscular paralysis also in Caucasians.

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Sommario Abbiamo presentato il caso di un uomo italiano di 21 anni affetto da tetraparesi ipokaliemica ad esordio acuto. Alla prima osservazione in pronto soccorso il paziente presentava agitazione, tachicardia (140/min) e ipertensione sistolica (180/70 mm Hg). Era ipotonico e incapace di fettere gli arti inferiori contro una lieve resistenza. I rif essi osteotendinei risultavano ineccitabili. Circa un'ora dopo il deficit di forza si estendeva agli arti superiori. Gli esami biochimici evidenziavano una severa ipopotassiemia (2.1 meq/l). Dopo infusione e.v. di 140 meq di potassio fosfato il deficit di forza migliorava rapidamente, e dopo circa 12 ore il paziente era in grado di camminare correttamente. Le indagini successive documentarono un morbo di Graves misconosciuto. La paralisi ipokaliemica tirotossica è stata descritta quasi esclusivamente nell'Asia orientale; questo caso, insieme a pochi altri riportati, dimostra the è da cosiderarsi causa di paralisi faccide acute anche nei caucasici.

     

Enterogenous cyst as a rare cause of hemifacial spasm

Hemifacial spasm (HFS) is most frequently caused by vascular compression of the facial nerve at the root exit zone of the brain stem, but there are other rare causes. An enterogenous cyst (EC) is a rare congenital lesion which occurs rarely in the cerebellopontine angle. The authors report a patient with HFS from an EC and review unusual causes of HFS.     

Popliteus muscle hemorrhage as a rare cause of a proximal tibial neuropathy

Proximal tibial neuropathy is an uncommon focal mononeuropathy that is most often caused by trauma, ischemia, or neoplastic infiltration or compression of the tibial nerve. We report a patient who presented with a tibial neuropathy following a leg injury, which initially mimicked a lumbosacral radiculopathy but which was the result of a proximal tibial neuropathy. Electrophysiologic studies confirmed a proximal tibial neuropathy and MRI revealed a popliteus muscle hemorrhage with mass effect on the tibial nerve. Following conservative management the patient had little recovery of function after 15 months.     

Melkersson-Rosenthal syndrome as a rare cause of recurrent facial nerve palsy

Melkersson-Rosenthal syndrome is a rare cause of recurrent facial nerve palsy. The syndrome is classically characterized by a triad of signs consisting of facial edema, recurrent peripheral facial nerve paralysis, and congenital fissured tongue, although it may also present in a mono- or oligosymptomatic form. The paper presents a 24-year-old woman with right-sided peripheral facial nerve palsy and a history of left-sided episodes of facial nerve palsy. Magnetic resonance angiography of the cerebral vessels suggested compression of the right seventh and eighth cranial nerves by a vascular loop. The authors describe the patient with a complete picture of Melkersson-Rosenthal syndrome and discuss the diagnosing process and treatment.     

Magnesium deficiency as a cause of acute intractable seizures

Summary Clinical and experimental investigations have shown that magnesium depletion causes a marked irritability of the nervous system, eventually resulting in epileptic seizures. Although magnesium deficiency as a cause of epilepsy is uncommon, its recognition and correction may prove life-saving. Two case reports are presented which emphasize the importance of recognizing hypomagnesaemia in patients with acute intractable seizures.     

The SCA12 mutation as a rare cause of spinocerebellar ataxia.

BACKGROUND: Spinocerebellar ataxias are a group of phenotypically and genetically heterogeneous disorders characterized by progressive degeneration of the cerebellum. The expansion of a CAG repeat upstream of the PP2APR55beta gene has been recently reported as a novel cause of a dominantly inherited ataxia (SCA12) in a kindred with limb tremor as an early feature. OBJECTIVE: To explore the relative frequency of SCA12 among familial and sporadic spinocerebellar ataxias in an ethnically diverse patient population. METHODS: We used polymerase chain reaction to analyze CAG repeat size in a series of patients presenting to an ataxia clinic in California. RESULTS: The SCA12 expansion was not detected in any of the cases investigated. The largest allele found had 22 repeats, a finding within the proposed nonpathogenic range. Distribution of repeat size and heterozygosity were similar to that described previously. CONCLUSIONS: These results, coupled with findings in other populations, indicate that the SCA12 mutation is a rare cause of spinocerebellar degeneration. Diagnostic testing for SCA12 should be considered in patients with cerebellum disorders and an atypical clinical phenotype, especially when tremor is initially present.     

Cerebral contusion as a rare cause of foot drop: case report

A 74-year-old woman, taking anticoagulant therapy for chronic heart failure, presented to our emergency room with left dorsiflexion weakness 8 hours from after multitrauma. A detailed neurological examination revealed only 0/5 strength in the left foot dorsiflexion without any upper motor neuron signs. While there was no spinal cord pathology detected, cranial computed tomography demonstrated a lesion in the right parasagittal localization consistent with hemorrhagic contusion. Clinical follow-ups showed an improvement in neurological findings with muscle power of 3/5 in day 5 and 5/5 in day 45 of admission. The parasagittal region has a foot localization in the homonculus and lesions in this area can rarely present with the foot drop sign. Thus, parasagittal region lesions should always be kept in mind in foot drop cases.     

Multiple sclerosis as a cause of the acute vestibular syndrome

Multiple sclerosis (MS) causes dizziness and vertigo. Reports suggest responsible lesions are often in the intra-pontine 8th nerve fascicle. We sought to determine frequency and clinical features of demyelinating acute vestibular syndrome (AVS). This is a prospective observational study (1999–2011). Consecutive AVS patients (vertigo, nystagmus, nausea/vomiting, head-motion intolerance, unsteady gait) with a risk for central localization underwent structured bedside examination and neuroimaging. When applicable, we identified MS based on clinical, imaging, and laboratory features. Of 170 AVS presentations, 4 % (n = 7) were due to demyelinating disease. Five had an acute MS plaque likely responsible for the clinical syndrome. Lesion location varied—1 medulla; 1 inferior cerebellar peduncle; 1 middle cerebellar peduncle; 1 posterior pontine tegmentum; 1 in the intrapontine 8th nerve fascicle; 1 superior cerebellar peduncle; 1 midbrain. Only two had a lesion in or near the intra-pontine 8th nerve fascicle. Three were first presentations (i.e., clinically isolated demyelinating syndrome), while the others were known MS. All had central oculomotor signs. In two patients, the only central sign was a normal horizontal head impulse test (h-HIT) of vestibular function. All patients improved with steroid therapy. Demyelinating disease was an uncommon cause of AVS in our series. Symptomatic lesions were not restricted to the 8th nerve fascicle. Five patients had relatively obvious oculomotor signs, making differentiation from vestibular neuritis straightforward. Two patients had unidirectional, horizontal nystagmus that followed Alexander’s law and was suppressed with fixation (true pseudoneuritis). The presence of a normal h-HIT in these suggested central localization.     

Thyroid stimulating hormone microadenoma as a rare cause of thyrotoxicosis amenable to surgical cure

Hyperthyroidism due to a thyroid stimulating hormone (TSH) pituitary adenoma is rare. We report a 29-year-old woman with thyrotoxicosis and elevated serum 3,5,3',5'-tetraiodothyronine and TSH levels that resolved after a transsphenoidal excision of the detected TSH pituitary adenoma. The diagnosis and management options in such patients are reviewed.     

EEG findings in Konzo: a spastic para/tetraparesis of acute onset.

EEGs were recorded on 21 konzo subjects (median age 17 years) and 13 of their close healthy relatives (median age 41 years). Konzo subjects were clinically selected and classified according to the WHO criteria. Standard waking EEG recordings were performed according to the International 10-20 System. Slowing of the background activity with theta activity was the most common abnormality (57%). The more clinically severely affected the konzo subject, the more often generalized EEG abnormalities were seen. None of these abnormalities were considered specific for konzo nor related to the duration of the disorder; however, the findings indicate involvement of the cerebral cortex.     

Churg-Strauss, a rare cause of intracerebral haemorrhage

Intracerebral haemorrhage (ICH) is a common and devastating condition. We describe a 43-year-old man with the rare Churg-Strauss syndrome, which was diagnosed after he presented with an ICH. He was managed initially neurosurgically with clot evacuation and craniectomy. Post-investigation and diagnosis of Churg-Strauss, treatment was with high dose methylprednisolone (then oral prednsiolone) and intravenous cyclophosphamide. Clinicians should be aware that Churg-Strauss syndrome can, rarely, be a cause of ICH. Given its high morbidity and mortality, it is important that patients are diagnosed promptly and managed appropriately.