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Alice in Wonderland syndrome is a disorienting neurological condition that affects human perception to the senses of vision, hearing, touch, sensation, and the phenomenon of time. Individuals affected with Alice in Wonderland syndrome can experience alterations in their perception of the size of objects or their own body parts, known as metamorphopsias. It is known to occur in conditions including migraine, epilepsy, and certain intoxicants and infectious diseases. The name refers to Lewis Carrol's well-known children's book Alice's Adventures in Wonderland, in which the title character experiences alterations of sensation in which she felt that her body had grown too tall or too small, or parts of her body were changing shape, size, or relationship to the rest of her body. The syndrome was described in 1952 by Caro Lippman, and given its name in 1955 by John Todd. The metamorphopsias characteristic of this condition are also sometimes referred to as Lilliputian hallucinations, a reference to the fictional island of Lilliput in the novel Gulliver's Travels, written by Jonathan Swift in 1726. As such, many literary and medical publications have roots in the description of this syndrome. The purpose of this review is to summarize the literary and historical significance of Alice in Wonderland syndrome, as well as to provide the reader with a medical overview of the condition.

Fredrick E. Batten defined the pathology of subacute combined degeneration and made seminal observations about myasthenia gravis, poliomyelitis, and infantile neuronal lipofuscinosis.Fredrick Eusta...

We report a case of a patient with history of alcohol abuse, treatment for hepatitis C and repeated strenuous physical activity who developed severe muscle pain and weakness during statin therapy. The symptoms persisted after discontinuation of the drug. The diagnosis of myopathy was made clinically and by electromyography. As his symptoms persisted a muscle biopsy was performed which showed inclusions consistent with hyaline inclusions. Hyaline inclusion myopathy is discussed in the context of this case with review of the literature.

Charles Karsner Mills (1845–1931), perfected methods to use electrical stimuli of the cerebral cortex of alert humans to localize sites of their seizures. Mills meticulously recorded the neurological examination of patients and then correlated those findings with their brain autopsies to localize functions in human brain and spinal cord. He described a form of transcortical motor aphasia and effective methods to rehabilitate aphasic patients. Mills wrote The Nervous System and Its Diseases .

A 74-year-old man had diplopia, painful right ophthalmoplegia, proptosis, conjunctival injection, and facial skin lesions. Magnetic resonance imaging (MRI) revealed infiltration of the right intraorbital adipose tissue. Lesions were mixed low- and high-signal on T2-weighted images and enhanced on fat-suppressed T1-weighted postcontrast images. A skin biopsy revealed numerous noncaseating granulomas consistent with sarcoidosis. Treatment with corticosteroids and chlorambucil led to a full clinical recovery. Sarcoidosis should be considered in the evaluation of orbital pseudotumor in elderly patients, even if no systemic manifestations of sarcoidosis are present.

We compared the activity of Ib spinal interneurons in five patients with progressive supranuclear palsy (PSP) with six age-matched control subjects. Stimulation of the medial gastrocnemius nerve at motor threshold intensity activated Ib afferents that in turn inhibit H reflexes from the soleus muscle. Maximum inhibition occurred at interstimulus intervals of 6 and 8 ms for both control subjects and PSP patients and was significantly greater in the PSP patients. Increased Ib activity of PSP patients may be caused by loss of inhibition of Ib interneurons through degeneration of the medullary reticulospinal pathway. The corticospinal pathways, unopposed by the medullary reticulospinal tract, may excessively activate Ib interneurons.

ROSWELL PARK, M.D., (1852-1914) is remembered for founding the world's first cancer institute that now bears his name a century ago, The Roswell Park Cancer Institute, and for an unfortunate association with the mortal wounding of President William McKinley in Buffalo, NY, in 1901. Park's accomplishments as a pioneer American neurosurgeon have been overlooked. After Park was appointed as Chair of Surgery at the University of Buffalo in 1884, he became the first American surgeon to precisely localize and remove a posttraumatic epileptic focus in the absence of external scars in 1886. Park introduced American physicians and surgeons to David Ferrier's research on localization of cerebral cortical function and Victor Horsley's techniques for extirpating epileptic foci. In 1895, Park became the first American surgeon to successfully treat spina bifida. In the same year, he wrote the first American monograph on surgery of the head. Park's case reports of successful operations on patients deemed almost incurable reveal boldness and ingenuity. Park's untimely death truncated a promising career.

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We bring to reader's attention the book Medical-Moral Account of the Most Troublesome and Rigorous Illness of Epilepsy, written in 1754 by the Mexican physician Pedro de Horta. This book is the earliest text on epilepsy written in the New World. We examine Pedro de Horta's medical and religious concepts of epilepsy and analyze concepts of epilepsy in Hispanic communities during the eighteenth century.

• From biblical times to 1886, tremors were partially characterized by patterns of activation and associated disease states. William Richard Gowers and Frederick Peterson devised methods to determine the frequencies of the tremors of Parkinson's disease, alcoholism, hysteria, and other diseases. Their values concur closely with the contemporary ranges of 4.0 to 7.0 Hz in Parkinson's disease and 8.1 to 11.0 Hz in chronic alcoholism. This review properly credits these authors' pioneer investigations made with simple instruments a century ago.

Background Oculomotor nerve paresis may have relatively benign but also life-threatening causes. Distinguishing between these is of great clinical importance. Objective To reveal a potential pitfall of the clinical evaluation of oculomotor nerve paresis. Patient Single case observation. Results A 56-year-old man had fluctuating diplopia and fatigable ptosis, promptly relieved by intravenous edrophonium, leading to the diagnosis of ocular myasthenia gravis. His pupillary function was intact. A few days after the initial diagnosis, he suffered a subarachnoid hemorrhage secondary to the rupture of a basilar artery aneurysm. His ocular symptoms were related to aneurysmal oculomotor nerve compression. Conclusion Patients with oculomotor nerve dysfunction need more detailed evaluation because the underlying cause cannot be safely determined on a clinical basis.

The two most common phenotypes of X-linked adrenoleucodystrophy are the childhood cerebral form and adrenomyeloneuropathy, which occurs mainly in adults and affects the long tracts in the spinal cord most severely.1 Most patients with the cerebral forms have an inflammatory demyelinating process, while the principal pathology of adrenomyeloneuropathy is a non-inflammatory distal axonopathy, although 30% of patients with adrenomyeloneuropathy also develop some degree of inflammatory brain pathology.2 All forms of X-linked adrenoleucodystrophy are caused by a defect in the gene ABCD1 which codes for the peroxisomal membrane protein ALDP and is associated with the abnormal accumulation of very long chain fatty acids. Most patients with X-linked adrenoleucodystrophy have primary adrenocortical insufficiency. Although adrenal hormone treatment is considered mandatory and may be life saving, most investigators have expressed the opinion that it does not alter neurological status. We report a patient with a variant of adrenomyeloneuropathy in whom adrenal hormone replacement therapy improved neurophysiological function and clinical status. A 39 year old man was evaluated for adrenoleucodystrophy at the Kennedy–Krieger Institute (KKI) in 1985, because his nephew had been diagnosed with childhood onset adrenoleucodystrophy. The nephew died aged nine years and had necropsy confirmation of the diagnosis. Our …

The cerebellar examination evolved from observations of experimental lesions made by neurophysiologists and clinical descriptions of patients with trauma to the cerebellum. At the beginning of the 19th century, neurophysiologists such as Luigi Rolando, Marie-Jean-Pierre Flourens, and John Call Dalton, Jr. ablated portions of the cerebellum of a variety of animals and observed staggering gait, clumsiness, and falling from side to side without loss of strength. They concluded that the cerebellum coordinated voluntary movements. In 1899, Joseph Francois Felix Babinski observed that patients with cerebellar lesions could not execute complex movements without breaking down into their elemental movements and described the defect as dysmetria. In 1902, Babinski coined the term dysdiodochokinesis to describe the inability to perform rapid execution of movements requiring alternate contractions of agonist and antagonist muscles. Gordon Holmes in 1904 described the phenomena of rebound, noting that if a limb ipsilateral to a cerebellar lesion is suddenly released from tension, the appendage will flail. In 1917, Gordon Holmes reported hypotonia and dysmetria in men wounded by gunshot wounds to their cerebellum. These observations were rapidly included in descriptions of the cerebellar examination in popular contemporaneous textbooks of neurology. Modern observations have demonstrated that the cerebellum influences such cognitive functions such as planning, verbal fluency, abstract reasoning, prosody, and use of correct grammar.

Gordon Morgan Holmes, MD, MRCP was an Irish born neurologist who received his medical education at Trinity College, Dublin, Ireland. He was trained in neuroanatomy and neuropathology at the Senckenberg Institute, Frankfort-Am-Main by Ludwig Edinger. He then returned to serve as a Registrar (House Officer) mentored by Richard Gowers and John Hughlings Jackson at the National Hospital, Queen Square, London. He collaborated with Thomas Granger Stewart in describing the loss of recoil in patients with cerebellar hemispheric tumors in 1904. Volunteering in 1914 for frontline hospital duty, he examined soldiers who had injuries to their occipital area causing hypotonia, dysmetria, staggering gait, and falling to the side ipsilateral to their injured cerebellar hemisphere. Holmes discovered that increasing the pace of the finger-nose manuever and applying slight resistance to a moving limb attenuated the dysmetria. Continuing observation of these patients afforded him to describe the evolution of their injuries to include increasing tremor and decreasing hypotonia. Holmes first attached levers to the limbs of hispatients to record their movements on a moving smoked paper kymograph. In 1939 he published photograh tracings made by low mass minature light bulbs attached to ataxic limbs that showed thehpometira and hypometria of their movements ipsilateral to their damaged cerebellar lobes. Holmes made sigficant contributions to understanding of the physiology of the human cerebellum.

Neurologic manifestations of vitamin B12 deficiency are protean, including neuropathy, depression, and dementia. We present evidence to dispel confounding myths about vitamin B12 deficiency. Hematologic indices are normal in up to 30% of patients with vitamin B12 deficiency, and results of the Schilling test may be normal in patients with symptoms of deficiency. Isolated neuropathy or myelopathy may occur independently, but often appear concurrently. The neuropathy is primarily axonal and predominantly sensory. Myelopathy is caused by demyelinated areas in posterior and lateral columns. After therapy, recovery from neuropathy is incomplete or may extend for several years. Vitamin B12 replacement should not be withheld from patients with borderline vitamin B12 levels, since the consequences of allowing myelopathy, neuropathy, dementia, and mental disorders to worsen clearly outweigh any disadvantage of therapy.

A hypertensive man had a long standing history of contumacious hyperpathia in the right upper extremity, resistant to medical therapy, secondary to a lacunar infarct in the left thalamus. A second cerebrovascular accident caused a small lesion in the left corona radiata, interrupting the thalamoparietal interconnections, and terminated the pain instantly. Interruption of the subcortical parietal white matter may more effectively control pain than cortical lesions. A few surgeons have successfully treated rebellious chronic pain with stereotaxic operations in the corona radiata, resulting in lesions very similar to our patient's. This overlooked and nearly forgotten technique may still have value in treating selected cases.

Simultaneous occurrence of a cerebral arteriovenous malformation and a primary brain tumor is rare. A case of a left occipital meningioma and a right parietotemporal arteriovenous malformation is reported. Clinical, radiological, and postmortem findings are described. Thirty previous reports of arteriovenous malformations associated with primary brain tumors are reviewed. In 18 cases the two lesions were intermixed or in close proximity. This spatial relationship between the lesions suggests more than a coincidential association. Several hypotheses are proposed to explain common causal connections.

Reports of the incidence and severity of physiological abnormalities of vitamin B12 deficiency sharply differ. To resolve these controversies we performed evoked response and nerve conduction studies in 10 males with vitamin B12 deficiency. The minimum criteria for this diagnosis were diminished position and vibration sensation and vitamin B12 levels less than 220 pg/ml. We found normal BAERs in 9/10 patients. The central interpeak latencies of the median and peroneal SERs were abnormal in 4/10 and 8/8 patients, respectively. The VERs were abnormal in 7/10 patients. We found absent or diminished amplitudes of sural sensory and peroneal motor action potentials in 8/10 patients. Abnormal peroneal F response chronodispersion was present in 7/10 patients. Nerve conduction velocities (NCV) were normal in 9/10 patients. EMG demonstrated abnormalities consistent with denervation in lower extremity muscles in 8/10 patients. These studies present a profile of vitamin B12 deficiency: essentially normal BAERs, mildly abnormal VERs and median SERs, and markedly abnormal peroneal SERs. NCV studies are consistent with sensory-motor axonopathy.

A patient with a dominantly inherited form of Kuf's disease and an associated occipital astrocytoma is presented. This is the first reported case in which the diagnosis of Kufs' disease was made by a cortical biopsy several years before its expected clinical onset. The nosology of this disease, and its clinical, genetic, and histopathological characteristics are discussed. The establishment of an early diagnosis by cortical biopsy and its implications are considered.

Three patients with subacute combined degeneration of the spinal cord due to vitamin B12 deficiency showed a characteristic pattern of abnormalities on neurophysiological tests which corresponded to the clinical signs and symptoms. Peripheral nerve conduction studies showed an axonal degeneration peripheral neuropathy. Brain stem auditory evoked responses were normal; visual evoked responses were mildly abnormal and somatosensory evoked responses were moderately abnormal. These studies shed light on the pathology of this disorder and can aid in its diagnosis.

Computed axial tomography is of great value in lesion localization in patients with aphasia. We discuss a patient with alexia without agraphia where computed axial tomography was of aid in correlating the anatomical lesion with the functional deficit.

This case report describes the fluorescein angiography, the radioactive scintigraphy, and the pathologic findings of an intraocular metastasis from bronchogenic carcinoma which occurred in a 64-year-old white man. In spite of extensive studies, no definitive differential diagnosis between primary and secondary eye lesion could be established before enucleation for intractable pain. This case illustrates that presently available methods are not adequate for correct differential diagnosis in such cases.

A 63-year-old man developed headache, left hemiparesis, and progressive visual loss. Craniotomy demonstrated reticulum cell sarcoma within the right temporal lobe. Necropsy revealed extensive involvement of optic nerves by primary RCS. Autopsy suggested that local compression of optic nerves by tumor cell infiltrates may produce atrophy in addition to sustained, generalized elevation of intracranial pressure.

To the Editor.— Troncoso et al and Rees (Archives1979;36:168-169 and 1980;37:397) have reported prolonged latencies of the major positive component (P 2 ) of the visual evoked response in patients who have subacute combined degeneration resulting from pernicious anemia. Our experience with more complete neurophysiologic testing reveals a distinct pattern suggestive of subacute combined degeneration, which correlates well with the clinical features and the known pathologic finding. 1 The visual evoked response is not the most sensitive parameter. One of the three patients described and one additional patient seen since that time have shown normal visual evoked responses. The most sensitive parameter, which has been abnormal in all cases, is the somatosensory evoked response. A full battery of studies showing an axonal degeneration peripheral neuropathy, normal brainstem auditory evoked responses, mildly abnormal visual evoked responses, and clearly abnormal somatosensory evoked responses paint a picture that is compatible with the diagnosis

This communication describes a rare condition, unsuspected epidural abscess, present during an otherwise uncomplicated spinal anesthesia and emphasizes the absolutely essential role of postmortem examination in establishing both the cause and nature of concurrent but not causally related complications. Without postmortem examination the actual pathological condition would have been overlooked, and the information available prior to autopsy would have incriminated the unrelated procedure of spinal anesthesia. Report of a Case.— An 87-year-old woman required amputation of her left leg for diabetic gangrene of the heel and surrounding cellulitis. The patient was a known diabetic for 35 years, treated initially with insulin and more recently with tolbutamide (Orinase). Four years earlier, the right foot had been amputated for a similar condition. There was also a history of arteriosclerotic heart disease. At the time of admission, three weeks before operation, significant findings were blood pressure, 110/70 mm Hg; temperature, 101 F (38.3